Leiomyosarcoma is a malignant mesenchymal tumor derived from the smooth muscle, it represents approximately 7% of all soft tissue sarcomas. Male genitourinary leiomyosarcomas are rare (Abdullazade et al., 2013 [1]). Primary testicular leiomyosarcoma is an exceptional entity with only 30 cases reported in the literature (Giridhar et al., 2011). Due to its rarity, additional studies are necessary to better define the optimal therapeutic management. METHODS We report a case of a 42-years-old male diagnosed in the urology department A of the University Hospital Ibn Sina in Rabat who complains of testicular swelling. The anatomopathological examination and immunohistochemical study revealed a leiomyosarcoma therefore, a radical inguinal orchiectomy with a primary ligation of the spermatic cord was performed for diagnostic and therapeutic purposes. The assessment of extension did not reveal any lymph node location or secondary appearance thus the decision of the multidisciplinary meeting opted for regular cancer check-ups without adjuvant treatment. CONCLUSIONS The actual etiology of testicular leiomyosarcoma is still unknown added to its clinical presentation and radiological results that are non-specific. CONCLUSIONS Leiomyosarcoma of the testis is a very rare tumor and its clinical and radiological presentation remains similar to other testicular malignancies.