Cutaneous metastases from internal malignancies are uncommon. Furthermore, cutaneous metastases from cholangiocarcinoma are extremely rare. Here we report a case of two patients with distant cutaneous metastases of cholangiocarcinoma: 1) a 66-year-old man who presented with a solitary, erythematous nodule on the scalp and 2) a 44-yearold man who presented with multiple, erythematous nodules on the scalp, the chest wall, and the back. In both cases, the erythematous nodules were the first clinical signs of cholangiocarcinoma. Histopathological analyses of skin biopsy specimens of the two patients revealed adenocarcinomas with features similar to the original cholangiocarcinoma. Two cases of cholangiocarcinoma in which metastatic skin nodules appeared as the fi rst sign of the disease are reported here, with a review of the relevant literature. (Gut Liver 2011;5:100-104)