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We present a case of a 44 year-old man with histiocytic sarcoma arising from the rectum. Histiocytic sarcoma is a rare type of malignant lymphoma, comprising less than 0.5% of all non- Hodgkin’s lymphomas (NHLs). In this case, the tumor was a polypoid mass, measuring 2.5× 2 cm. On microscopic examination, it was composed of many bizarre multinucleated cells and sheets of xanthomatous histiocytes. There were also areas with spindle cells arranged in a storiform pattern. Extensive metastases to regional lymph nodes were found, and combined adjuvant chemotherapy was administered after complete resection.