Retroperitoneal angioleiomyoma is a benign mesenchymal neoplasm that is composed of smooth muscle cells and thick-walled vessels. In a 36-year-old patient a retrouterine and retroperitoneal tumor, 70 x 65 x 50 mm in size, was discovered during a surgical procedure due to uterine myoma. The tumor had a soft consistency and was completely removed. Histopathology showed features of angioleiomyoma. Angioleiomyoma is a rare benign entity; hence a benign course and good prognosis are expected.