Paratesticular tumors are extremely rare, with paratesticular rhabdomyosarcoma being the most common finding. A 6-month-old boy presented with an asymptomatic, right intrascrotal mass whereby the testicle was surrounded by a friable lipomatous tumor. Frozen section revealed an inflammatory process, negative for malignancy. Tumorectomy with vaginal resection was performed, maintaining the testicle and excretory ducts. Histopathologic findings showed a juvenile xanthogranuloma, a non-Langerhans histiocytosis commonly described in infants in the skin and skeletal muscle. The patient is doing well 2 years after surgery and is the first such case reported in the literature with successful conservative treatment.