A 39-year-old Sinhalese woman presented with obstructive jaundice of 3 months’ duration. She had deeply icteric sclera with evidence of pruritus. An abdominal examination revealed mild hepatomegaly. Serum bilirubin, alkaline phosphatase and gamma-glutamyl transferase levels were significantly elevated while the transaminases were moderately high. An ultrasound examination was suggestive of a cystic lesion in segment IV of her liver with dilated intrahepatic ducts. A triphasic computed tomography (CT) scan revealed a non-enhancing cystic lesion on segment IV with intrahepatic duct dilatation. A subsequent magnetic resonance imaging (MRI) scan of her liver and magnetic resonance cholangiopancreatogram (MRCP) confirmed the cystic lesion in her liver and a mass lesion occupying the common hepatic duct and proximal common bile duct up to the level of her duodenum. Her serum carbohydrate antigen (CA) 19–9 was over 1000U/mL (normal <40U/mL). In the interim period she underwent an endoscopic retrograde cholangiopancreatogram (ERCP) and temporary stent placement to relieve biliary obstruction. Brush cytology obtained during ERCP did not reveal abnormal cells. We could not arrive at a diagnosis with the available evidence. As definitive treatment she underwent left hemihepatectomy and excision of extrahepatic bile duct and reconstruction by hepaticojejunostomy. Examination of the specimen revealed a cystic lesion located in segment IV of her liver with extension of a solid mass along the duct of segment IV, up to her common bile duct completely obstructing the extrahepatic biliary system. A histological examination of multiple cross-sections of the specimen revealed a cystic space lined by a simple mucin-secreting columnar to low-cuboidal epithelium. The subepithelial tissue resembled ovarian stromal tissue. None of the examined sections revealed papillomatosis or nuclear atypia. No foci of malignant transformation were observed. She had an uneventful recovery. She was symptom free at her 3-month postoperative follow-up visit with no clinical or ultrasonic evidence of recurrence.