A soft tissue tumor causing left lower extremity swelling in a 43 years old female was detected in the left inguinal region and resection was performed at the Department of Cardiovascular Surgery of Semmelweis University of Medicine, Budapest. Histologic examination revealed primary leiomyosarcoma of vascular origin. On the base of this case attention is called to this rare disease, which has poor prognosis. Early diagnosis and complete resection plays key role in the treatment of leiomyosarcoma and may improve survival.