Paratesticular Rhabdomyosarcoma is a rare mesenchymal tumor. The Alveolar variant is the one with the worse prognosis. It comprehend 20% of cases. The treatment is multimodal combining surgery, chemotherapy and radiotherapy. Depending on the extent of disease and the staging group, the approach of treatment is different. Here we report the case of an alveolar Paratesticular rhabdomyosarcoma and review the literature regarding this unusual clinical entity.