In children, paratesticular tumors represent approximately 1-2% of solid tumors, with an incidence of 0.5-2 per 100,000 children. The most frequent anatomopathological type in children is rhabdomyosarcoma, and other types of paratesticular tumours are exceptional. In most cases, these tumors are unrecognized and sometimes initially misdiagnosed. These tumors require multidisciplinary management to improve patient survival. Treatment is essentially surgical, irrespective of tumour type and stage, and often requires chemotherapy depending on the tumour and tumour extension.