Background : Malignant hepato-biliary disease is known to be the most serious complication in patient with hepatolithiasis. The development of cholangiocarcinoma in patients with hepatolithiasis is associated with poor long-term outcomes.

Methods : We present a case of concomitant cholangiocarcinoma with hepatolithiasis in a 46-year-old woman. The tumor and the intrahepatic stone were detected in diagnostic work up due to her complaint of recurrent abdominal pain.

Results : Right hemihepatectomy and cholecystectomy was performed in this patient. A histopathologic of the specimen revealed a cholangiocarcinoma and chronic cholecystitis. Several risk factors known for this malignancy such as primary sclerosing cholangitis, parasitic infection, history of viral hepatitis infection and cirrhosis are not found in the patient.

Conclusions : This report describes hepatolitihiasis as a risk factor for cholangiocarcinoma, and also emphasizes the evaluation and surgical treatment for the malignant tumor.