A case of hilar biliary cystadenoma with elevated IgG4 levels.

Cholangiocytic adenoma in the hilar bile duct is rare, and elevated IgG4 at the same time is extremely rare. This situation has not been reported in the literature. Nonetheless, the current case involved hilar biliary cystadenoma with elevated IgG4 levels. A 66-year-old man presented at this hospital with dark tea-colored urine. Preoperative imaging studies suggested hilar cholangiocarcinoma. This case demonstrates the difficulty of preoperative diagnosis of benign hilar lesions and the rarity of two combined benign lesions. A point of contention is whether this case should be treated with surgery or hormone therapy.