Case Reports in Medicine preferably aﬀects male with a maximum age of around 25– 45 [1]. About 80% of patients show both intrahepatic and extrahepatic involvement; 20% showed only extrahepatic involvement [2]. It is likely an immune-mediated disease with a wide range of autoantibodies detected in which p-ANCA, having the highest prevalence and detected in our patient, occurs in 80% of patients. Although it does not correlate with the activity of PSC, it may draw attention to colon involvement [3]. For diagnostic imaging, the typical ﬁndings of ERCP and MRCP in PSC are pearl-string-like changes of the bile ducts with intermittent, diﬀusely distributed, multiple, and irregular strictures of diﬀerent length. Nowadays MRCP replaces the role of ERCP in the diagnosis of PSC because it is noninvasive with high sensitivity and speciﬁcity (both are greater than 80%) whereas ERCP can lead to potential serious complications such as pancreatitis and bacterial cholangitis [4]. As MRCP is not easily available in our centre, ERCP was performed instead, and the typical strictures and pruning of the biliary tree were demonstrated and two stones were found concurrently and removed uncomplicatedly in the same session. The liver biopsy of our case revealed the typical features of PSC. In fact, a liver biopsy is not required in the presence of typical cholangiographic features of PSC unless a small duct PSC is suspected because the disease localizes in intrahepatic ducts [5]. It was performed in our case because he was young with quite advanced laboratory parameters and had an elevated serum aminotransferase. Thus an accurate staging of the disease and exclusion of a PSC-autoimmune hepatitis (PSC-AIH) are warranted. A clinical entity called autoimmune pancreatitis (AIP-SC), characterized by a lymphoplasmacytic inﬁltrate around pan- creatic duct and elevated serum IgG4, can cause stricturing in intrahepatic and extrahepatic bile duct similar to that present in PSC. Distinguishing from PSC, both PSC-AIH and AIP- SC are responsive to corticosteroid [6]. PSC is strongly associated with inﬂammatory bowel disease (IBD) in which up to 80% are associated with ulcerative colitis (UC), and only 10% Crohn’s disease with the latter is usually diagnosed before PSC [7]. As IBD in PSC may be asymptomatic, and therefore any newly diagnosed PSC should have full colonoscopy with biopsies. Although IBD may be diagnosed at any time during the course of PSC, the concomitant presentation like in our case is not typical [8]. There are several clinical and endoscopic features of IBD in PSC distinctive from ordinary IBD in which the former usually has more pancolitis, rectal sparing, and backwash ileitis. These mentioned features are present in our patient. In addition, IBD has more frequently quiescent and prolonged subclinical course [9, 10]. The medical therapy does not diﬀer from that of IBD without PSC. The indications for urgent surgery are acute severe colitis not responding to medical treatment, toxic dilatation, perforation, or hemor- rhage while the indications for elective surgery fall into two groups: failure of medical therapy and dysplastic/malignant change in the colon. Proctocolectomy with ileal pouchanal anastomosis is now the procedure of choice because it has the advantage of both removing the diseased colon and avoiding a permanent ileostomy. Patients with UC and PSC are at 3 higher risk of colorectal neoplasia compared with those with UC alone, with odd ratio 4.79 and predilection for right- side distribution [11]. Thus, surveillance colonoscopy with biopsies at up to two-year intervals is recommended. There is no eﬀective medical treatment of PSC; the routine use of ursodeoxycholic acid (UDCA) is not rec- ommended because of unclear beneﬁt and possible serious adverse eﬀects in high dose (28–30 mg/kg/day) [12]. Treat- ment with corticosteroid and other immunosuppressant agents does not show any beneﬁcial eﬀect. However, our case received UDCA of the dose around 15 mg/kg/day, and his serum liver ductal enzymes seemed improving, but the long- term eﬀect needs to be observed. The management of our patient should also include management of potential complications including portal hypertension, dominant strictures of bile ducts, gallbladder diseases, cholangiocarcinoma, and colorectal neoplasia. Concerning portal hypertension, about 40% newly diag- nosed cases have esophageal varices, and its management does not diﬀer from non-PSC patients [13]. The dominant stricture in PSC is deﬁned as a stenosis with a diameter of less than 1.5 mm in common bile duct or of 1 mm in intrahepatic ducts [14]. It happens in about 50% of patients during the followup, and the common presentations are jaundice, pruritus, right-upper quadrant pain, and elevated serum bilirubin. The management is to relieve biliary obstruction by endoscopic balloon dilatation with or without stent placement preceded by the brush cytology and biopsy at the stricture site to exclude a superimposed malignancy [15]. In an early case series, the gallbladder abnormalities are frequently observed, including gallstones (26%), PSC involving gallbladder (15%), and neoplasm of gallbladder (4%) [16]. Therefore, an annual ultrasound of biliary system is recommended to detect mass lesions in the gallbladder. In our case, the coexisting common bile duct stones might be formed in situ or migrated from the gallbladder. Lastly, PSC is a risk factor for cholangiocarcinoma, with 10% ten- year cumulative incidence [17]. It is a diﬃcult task to dis- tinguish it from the benign stricture. Because of lack of any diagnostic test, the diagnosis of cholangiocarcinoma relies on the following features: contrast-enhanced mass lesion in imaging, positive biopsy/cytology, or highly elevated CA 19-9 in case of borderline resulting from imaging and histol- ogy. For early stage of cholangiocarcinoma, surgical resection is indicated in patient of good liver functional condition while liver transplantation following neoadjuvant therapy is an option in case of poor liver reserve [18]. The natural course of PSC depends on the respective stage at the time of diagnosis, and its survival rate is around 60% after 6 years [19]. Because of the absence of eﬀective treatment available, there are several prognostic models to predict the clinical outcome, such as Mayo score, which is shown to be useful in predicting the clinical course particularly the early stage of PSC, and this score includes age, bilirubin, serum aminotransferase, albumin, and history of variceal bleeding [20]. Liver transplant indications for patient with PSC include liver failure and several unique indications such as intractable pruritus, recurrent bacterial cholangitis, and cholangiocarcinoma. The appropriate time