Angiolipoma is a common benign tumor with a char- acteristic vascular component that usually occurs in subcutaneous tissue. Although lipomas are frequently encountered at colonoscopy as submucosal tumors, an- giolipomas are rarely found in the gastrointestinal tract including the large intestine. Here we report a 77-year old Japanese man who underwent transanal resection of a tumor that was diagnosed tentatively as a leiomyoma. Histologically, the tumor consisted of mature fat cells and blood vessels. Immunohistochemically, the tumor cells were negative for c-kit and HMB-45, which are consis- tently expressed in tumors such as gastrointestinal stro- mal tumor and angiomyolipoma. The tumor was there- fore diagnosed as an angiolipoma. This is thought to be the first case report of a surgically resected angiolipoma of the rectum. Angiolipoma is a common benign tumor with a char- acteristic vascular component that usually occurs in subcutaneous tissue. Although lipomas are frequently encountered in colonoscopy as submucosal tumors, an- giolipomas are rarely found in the gastrointestinal tract including the large intestine. Here we report a 77-year old Japanese man who underwent transanal resection of a tumor that was diagnosed tentatively as a leiomyoma. Histologically, the tumor consisted of mature fat cells and blood vessels. Immunohistochemically, the tumor cells were negative for c-kit and HMB-45, which are consis- tently expressed in tumors such as gastrointestinal stro- mal tumor and angiomyolipoma. The tumor was there- fore diagnosed as an angiolipoma. This is thought to be the first case report of a surgically resected angiolipoma of the rectum.