Colonic small cell carcinomas (SCCs) are rare, aggressive tumors characterized by early metastasis and poor prognosis. We describe a 39-year-old man with a history of ulcerative colitis and left colon adenocarcinoma resected at an outside institution who presented with hematochezia and proctalgia. Work-up revealed left colon SCC with liver metastases. After his excellent response to neoadjuvant chemotherapy, we performed synchronous proctocolectomy and right hepatectomy. Final pathologic evaluation revealed colonic primary SCC and recurrent adenocarcinoma with metastatic SCC to the liver; lymph nodes were positive for metastatic SCC and adenocarcinoma. SCC recurrence ultimately developed in the liver. To the best of our knowledge, this is the second reported case of surgical management of SCC of the colon with liver metastasis and the first report of synchronous excision. Despite superb response to neoadjuvant therapy and young patient age, caution is needed in surgical resection for SCC of the colon given the high recurrence risk.