Ganglioneuromas are rare benign tumors originating from the sympathetic nervous system and neural crest cells. A 4-year-old girl presented with numerous urinary tract infections. Ultrasound and computed tomography revealed a large mass within the right kidney. A right nephrectomy and sampling of surrounding lymph nodes were performed. Pathology confirmed that the mass was a mature ganglioneuroma. The patient remains disease-free, more than 2 years after surgery. We present this rare case of renal ganglioneuroma as well as a review of the literature.