Hemangioendotheliomas are poorly differentiated neoplasms built from strands of endothelial cells and have a very narrow lumen filled with blood cells. Hemangioendotheliomas are commonly detected during the initial six months of life, concerning mainly soft tissues and skin. It is the most common bening liver cancer in children. In adults, hemangioendotheliomas are very rare findings, being mostly described in the liver, lungs, brain and bones. Hemangioendotheliomas of the large intestine are extremely rare in adults. Cancer developing there is of high degree of malignancy. The authors present the case of 68 year old patient in whom preoperative benign tumor with ulcers were diagnosed. Intraoperative macroscopic picture suggested malignant process. The patient underwent anterior rectal resection with regional lymphadenectomy. The postoperative histopathological evaluation with immunohistochemical studies angiosarcoma was diagnosed. A four year survival free of cancer was obtained, and the patient died due to myocardial infarct. Literature describes the short periods of survival of patients with vascular sarcoma of the colon. The most common reason for decease are metastases with massive haemorrhages. Bleeding tendency results from growing Kasabach-Merritt syndrome. It is characterized by profound thrombocytopenia and coagulopathy. The presented patient is a unique example of angiosarcoma with long period of survival.