Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare type of primary liver cancer comprising histopathological features of both hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC). Because of its rarity and controversial diagnostic criteria, it continues to be poorly understood with a lack of well-delineated treatment options for recurrent or metastatic disease. We report a case of cHCC-CC in a 31-year-old woman with no risk factors for HCC or CC with recurrent pulmonary metastasis treated with systemic chemotherapy.