Desmoplastic Small Round Cell Tumor (DSRCT) is an aggressive neoplasm that classically grows in peritoneal cavity and has poor prognosis. We describe a 13-year old boy with testicular DSRCT, treated with orchiectomy and chemotherapy and is free of disease 96 months after diagnosis. This is the fifth case of primary DSRCT of the testis described in literature. Moreover, we present a literature review of 28 cases of testicular and paratesticular DSRCT and discuss these locations as a prognostic factor.