Hilar cholangiocarcinoma, also known as Klatskin tumor, is the most common type of cholangiocarcinoma. It usually has a lymphatic spread and is rarely associated with an umbilical nodule, also known as Sister Mary Joseph nodule. We report a case of a 53-year-old Caucasian man with hilar cholangiocarcinoma. The patient had an inoperable tumor and was referred to our center for liver transplantation. Post liver transplantation, the patient presented with a recurrence of the carcinoma in the umbilical region. The patient was found to have Sister Mary Joseph nodule. It carries a poor prognosis, and our patient succumbed to the illness in four months. Cholangiocarcinoma carries a poor prognosis. Surgical resection and liver transplantation with neoadjuvant chemoradiation are the preferred treatment strategies. Association of cholangiocarcinoma with umbilical metastasis is rare, and our patient had an even rarer presentation in the form of recurrence with umbilical nodule post-liver transplantation. We want to increase the awareness of the rare presentation, association, and recurrence of hilar cholangiocarcinoma in the form of umbilical nodule post-liver transplantation.
