Objective: IgG4-associated inflammatory pseudo-tumor (IPT) is a novel clinico-pathologic entity characterized by intensive infiltration of IgG4-positive plasma cells, associated with systemic IgG4-related sclerosing disease. Many reports described IgG4-related IPT in various locations such as pancreas, salivary glands, liver, breast, lung and recently also ureter. Method: It is described a case of ureteral IPT with pathologic and immunohistochemical features of IgG4-related IPT, fibrohistiocytic type. Results: The study case is a 82-year-old female with severe stenosis of the left ureter and hydronephrosis, who underwent to nephroureterectomy and endoscopic resection of multiple lesions in the bladder. The histological examination showed transmural fibrosing inflammatory lesion of the affected ureteral wall, with abundant plasma cells intermixed with many histiocytes, lymphocytes, fibroblasts and scattered eosinophils. The majority of infiltrating plasma cells were positive for IgG4. Bladder lesions showed similar histological features. The diagnosis was IgG4-related IPT, fibrohistiocytic type. Conclusion: IgG4-related IPT of ureter is extremely rare, with only few cases reported in literature. Recognition of this entity is clinically relevant because this type of IPT can be treated with steroid therapy and may be associated with sclerosing autoimmune disease in other organs.