Proximal common bile duct adenomyoma is a rare benign tumor. It is more common in female and elderly. Itproduces obstructive jaundice mimic to cholangiocarcinoma. It is usually evaluated by liver function test, tumormarker (CA19-9), Magnetic resonance cholangiopancreatogphy (MRI-MRCP), and endoscopic retrogradecholangiopancreatography (ERCP) with biopsy. Confirmatory diagnosis is almost impossible before surgery.Confirmation is only possible after surgery and histopathological examination. Recently we have encountereda 31 years old man who presented with obstructive jaundice. He was evaluated as usual and diagnosed as acase of hilar cholangiocarcinoma (Bismuth type I). Extrahepatic excision, peri-choledochal hepatic, retro-duodenal lymph node excision was done and the operation was completed by Roux-en-Y reconstruction.Histopathological examination confirms the lesion as bile duct adenomyoma. We are going to report the caseas bile duct adenomyoma in a young male which is a very rare tumor for understanding its presentation,diagnosis and treatment in light of published literature.