INTRODUCTION: Intrahepatic cholangiocarcinoma (ICC) is a highly malignant cancer for which surgery is the only curative treatment. The prognosis of ICC is extremely poor, especially in cases of lymph node metastasis (LNM), owing to the high postoperative recurrence rate. Herein, we present a case of advanced ICC with a breast cancer susceptibility gene-2 ( CASE PRESENTATION: A 52-year-old woman was referred to our hospital and was subsequently diagnosed with bilateral breast cancer. Computed tomography (CT) and magnetic resonance imaging incidentally detected a liver tumor in the hilar region and lymph node enlargement in the hepatoduodenal ligament. A 19 mm tumor was observed in the area surrounded by the right and left branches of the portal vein and an abnormal portal branch of segment 7. Positron emission tomography-CT showed fluorodeoxyglucose uptake in the liver tumor, hepatoduodenal ligament lymph nodes, and bilateral breasts. A tumor biopsy showed a papillary tumor, and ICC was suspected. As ICC with LNM has a poor prognosis, neoadjuvant chemotherapy was planned. Genetic testing using a blood sample revealed a CONCLUSIONS: We encountered a patient with advanced ICC with a