Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the prerogative of the child and the young adult. On physical examination, a painless scrotal tumefaction is observed. His diagnosis is essentially provided by testicular ultrasound. His therapeutic strategy is codified and based on the combination of chemotherapy and surgical excision or radiotherapy. The precocity of the management and the accuracy of the extension and radiology tests are important for the vital and functional prognosis. We report a case of paratesticular rhabdomysarcoma of alveolar type, diagnosed in front of a large left painless scrotal mass, which has been evolving for less than one month in a 7-year-old child. An inguinal orchiectomy was performed. A histological examination of the excised tissue revealed an epididymal RMS of the alveolar type. Our patient had 9 chemotherapy sessions with vincristine, actinomycin C and cyclophosphamide. Each chemotherapy session took place over four days, with a 21-day cycle. He was evaluated for 4 years and demonstrated good clinical improvement. The experience gained from the index case is crucial for the management of patients with intratesticular rhabdomyosarcoma who must always ensure that the radical inguinal orchiectomy with lymph node dissection is covered by chemotherapy and/or radiotherapy. This will potentially reduce the chances of tumor recurrence and/or metastasis, thus improving patient prognosis.