Primary leiomyosarcoma of the testis is an extremely rare disease entity of the genito-urinary tract. Although its clinical presentation does not seem to differ from that of other testicular malignancies, the clinical stage with which patients have been treated, as reported in contemporary literature, has always been stage 1. Diagnosis is achieved by combining histologic and immunohistochemical findings. Histologic findings refer to the presence of a spindle cell component with nuclear pleomorphism, while immunohistochemical findings refer to its reaction to specific antibodies. Although the number of reported cases is not significant and the clinical and biological behaviour of these tumors are very hard to predict, we demonstrate that radical orchidectomy followed by surveillance appears to be the treatment of choice. Retroperitoneal lymphadenectomy, radiotherapy and chemotherapy do not seem to have any place in the treatment of this type of malignancy.