Background/Aim: The prognosis of cholangiolocarcinoma, a rare malignant liver tumor derived from hepatic progenitor or stem cells, is considered relatively good; however, it frequently recurs. We herein present the diagnosis, histological findings, and treatment of cholangiolocarcinoma. Case Report: A 65-year-old woman with a large liver tumor (70 mm in diameter) was referred. Hepatocellular carcinoma was suspected based on strong early enhancement and delayed washout by enhanced computed tomography. The patient underwent curative left tri-sectionectomy. Histologically, she was diagnosed with pure cholangiolocarcinoma in a slightly fibrous liver. Three metachronous recurrent lesions (all ≤10 mm in diameter) were found between fifteen and twenty months after the initial hepatectomy. One lesion and the remaining two lesions were treated with hepatectomy and radiofrequency ablation, respectively. Two years after the initial diagnosis, she was doing well without recurrence. Conclusion: Repeated hepatectomy and radiofrequency ablation might be useful for small intrahepatic recurrences of cholangiolocarcinoma.Cholangiolocarcinoma (CLC), previously named as cholangiolocellular carcinoma, is a rare malignant liver tumor arising from hepatic progenitor or stem cells; CLC is also defined as a variant of combined hepatocellular carcinomacholangiocarcinoma (cHCC-CCA) by the 2018 World Health Organization classification (1, 2). cHCC-CCAs comprising CLC in more than 90% of the entire tumor are defined as CLC. (3). Due to its low incidence of 0.56% in patients with resected primary liver cancer, the clinicopathological features of CLC are not well defined (4). Macroscopically, CLC mimics mass-forming type intrahepatic cholangiocarcinoma (ICC); however, its malignant features are less extensive compared to ICC (5). Long-term survival after liver resection is significantly higher in patients with CLC compared to those with mass-forming type ICC.Liver cirrhosis (27%) and chronic hepatitis with varying degrees of fibrosis (73%) were defined as the background liver conditions in a study of 30 patients with CLC (3). In another study, 76% of the 29 patients had chronic liver disease, which was significantly greater than 48% in those with mass-forming type ICC (6).In diagnostic imaging studies, CLC mimics mass-forming type ICC in the liver periphery and often shows clinical and imaging findings similar to those of hepatocellular carcinoma (HCC) (7-10). On arterial phase images, CLC appears as a mass with early and complete enhancement or with predominantly peripheral enhancement. Contrast medium retention in the lesion is observed in half of the patients (7-10).Standard treatment for ICC and cHCC-CCA, including CLC, is liver resection and systemic chemotherapy (11,12). In contrast, radiofrequency ablation (RFA) has not been recommended for ICC because of insufficient local control (13). Because of the lower malignant feature compared with ICC, RFA for CLC is considered to provide good local control and long-term survival (14).