the highest prevalence is seen in sephardic jews from iran and iraq that is 1:3000 who also often have an associated coagulation factor vii deficiency ( 14 ) . dubin johnson syndrome manifests with an intermittent jaundice in the first two decades of life . pregnancy or intake of oral contraceptive may provoke manifestation of the disease . except for the jaundice the patient is a 22-year old male that came to the office 3 year ago for the first time with fever , jaundice , fatigue and dark urine . he had no history of alcohol intake or any drug use ( herbal or chemical ) . liver function test were impaired and all serological studies was normal but havab igm was positive , the patient was being treated with the suspicion of acute viral hepatitis a ( table 1 ) . paraclinical evaluations from first presentation until present ast= aspartate aminotransferase ; alt= alanine transaminase ; alp= alkaline phosphatase ; inr= international normalized ratio ; alb= albumin ; lkm ab= liver kidney microsome antibody ; asma= anti - smooth muscle antibody ; ana= antinuclear antibody ; ab= antibody ; ag= antigen ; ig= immunoglobulin ; hbs= hepatitis b surface ; hbc= hepatitis b core ; hcv= hepatitis virus type c ; hiv= human immunodeficiency virus ; hav= hepatitis a virus ; tsh= thyroid stimulating hormone ; s.p= serum protein ; ema= endomysial antibody ; p - anca= perinuclear anti - neutrophil cytoplasmic antibody in the recent past 3 years total bilirubin has been fluctuates between 8.9- 13.5 mg / dl and the direct part between 7.4 - 9.5 mg / dl . despite the regression of symptoms and reduction of liver enzymes to the normal levels , the jaundice remained persistent ( table 1 ) . abdominal sonography in 3 years ago revealed nothing significant but mild hepatomegaly with heterogenous echo . recently , liver biopsy was done and containing a specimen consisting of two pieces of small creamy needle - shaped dark brown tissue totally measuring 3 cm in length and 0.1 cm in diameter . individual hepatocytes contain abundant course brown pigment granules especially in perivenular areas portal tracts show mild lymphocytic infiltration . he had one episode of upper gi bleeding that esophagogastroduodenoscopy revealed grade b esophagitis and small duodenal ulcer . in urine djs is listed as a rare disease by the office of rare disease ( ord ) of the national institutes of health ( nih ) ( 6 , 7 ) . in this situation the diagnosis is based on clinical and laboratory findings especially liver biopsy . despite the normal liver enzymes , only the bilirubin is higher than normal level that is mainly conjugated part . in liver biopsy the total coproporphyrin in urine is normal , but 85 - 90% of urinary coproporphyrin is type i , whereas in normal persons 75% of urinary coproporphyrin is type iii ( 7 , 9 , 10 ) . in our patient the jaundice was being provoked after a viral hepatitis and despite comprehensive work up , only direct hyperbilirubinemia was seen .
elevated serum level of bilirubin is a common manifestation which is occurred in several diseases . 
 hyperbilirubinemia can manifest either conjugated or unconjugated . 
 conjugated or direct hyperbilirubinemia usually are caused by hepatocellular diseases or cholestatic liver diseases . 
 merely conjugated hyperbilirubinemia is the main manifestation of two congenital syndromes , including dubin - johnson and rotor syndrome ; however it can be seen in some patients with recurrent benign intrahepatic cholestasis . 
 this article reports a patient with dubin- johuson syndrome as a benign and rare condition .