A 68-year-old woman presented with yellowish discharge oozing from a fistula opening in the upper epigastric area that had persisted for one month prior to her visit. The patient had undergone a left lateral segmentectomy of the liver ten years prior for treatment of intrahepatic duct (IHD) stones. An abdominal computed tomography (CT) scan showed focal stricture and proximal dilatation of remnant IHD and a 1 cm-sized rim-enhancing lesion located under the surgical bed of the abdominal wall surrounding the dilated remnant IHD. Despite conservative management including nasobiliary drainage, no further improvement was anticipated. Partial hepatectomy and fistulectomy were performed for pathologic diagnosis and treatment of the enhancing lesion. Histopathology revealed adenocarcinoma. In this case, cholangiocarcinoma might have arisen in association with IHD stones and then developed a choledocho-cutaneous fistula as a clinical manifestation.