Paratesticular sarcomas are rare. Most cases reported in the literature are depending on retrospective analysis of case reports, small series, literature reviews, and expert opinion and they show different outcomes depending on several variables. The majority indicate that this may be an indolent tumor with a potential for cure if treated early. We present a case of a 58 year old man with a history of painless enlargement of the right testis. The ultrasound showed an extratesticular lesion and a right excisional of the mass was performed. The pathological examination revealed a leiomyosarcoma, and was then completed with a right radical inguinal orchiectomy. A review of the literature regarding paratesticular sarcomas presentation, diagnosis and treatment is presented in this article.