Central biliary metastasis from colorectal cancer (CRC) is a rare manifestation. Given the rarity of the lesion, cases with central biliary metastasis from CRC pose challenges in identifying the proper treatment strategy. We present a case of stage IV CRC with synchronous multiple liver metastases and central biliary invasion. We also conducted a literature search and reviewed the reports on central biliary metastasis of CRC treated by major hepatectomy combined with extrahepatic bile duct resection. The patient underwent colon resection, partial hepatectomy, and transileocecal portal embolization (TIPE) as the first procedure, which was followed by an extended right hepatectomy and extrahepatic bile duct resection. Adjuvant chemotherapy was introduced, and the patient was doing well with no recurrence at 17 months after the first procedure. A liberal radical and aggressive resection in a multimodal treatment strategy could offer a favorable outcome in patients with a rare central biliary manifestation of CRC.