The authors report a case of follicular lymphoma of the testis in a 3-year-old boy. Follicular lymphoma of childhood is rare, accounting for less than 5% of all non-Hodgkin lymphomas in the pediatric population. Children with follicular NHL have typically been described as presenting with localized disease, with a preponderance of male patients. The authors conducted a comprehensive review of the literature and found that this patient is the only child reported to have localized follicular lymphoma of the testis successfully treated without systemic chemotherapy. In all previously reported cases, a favorable outcome was seen following surgical resection and chemotherapy. This patient has remained disease-free for more than 2 years after surgical resection alone. The successful outcome in this case following surgical resection, without postoperative adjuvant chemotherapy, suggests that localized follicular non-Hodgkin lymphoma in children may not be associated with occult systemic disease and that subsequent chemotherapy may not be necessary for cure.