Vascular leiomyosarcomas are extremely rare tumors and represent only 0.001% of all malignancies. Venous leiomyosarcomas occur five times more often than arterial ones, with 50% of them originating in the inferior vena cava (IVC). Arterial leiomyosarcomas are most commonly encountered in the great vessels with less than 50% of them occurring in the peripheral circulation. A total of only seven cases of arterial leiomyosarcomas involving the femoral artery have been reported in literature to date. We report a case of an arterial leiomyosarcoma involving the profunda femoris artery, and provide a comprehensive review on peripheral arterial leiomyosarcomas-distribution, clinical presentation, radiological and histological diagnosis, staging, and treatment.