Primary leiomyosarcoma of the penis is very rare. Since 1930, only 53 cases have been reported in the international literature. We describe a case of primary leiomyosarcoma of the penis diagnosed in 2008 in a 63-year-oldman, presented to our department with a firm-fibrous mass involving the ventrolateral side of the shaft, treated with local excision and 3 times locally recurred. We also attempt a review of the literature regarding evolution, progression, differential diagnosis and possible treatment of this disease.