We report a rare case of an intrarenal teratoma in a 6-month-old male. Following biopsy, he was successfully managed with a primary nephrectomy. This patient presented as would a Wilms tumor. According to SIOP guidelines, the diagnosis of Wilms tumor is made on clinical and imaging information only, prior to neoadjuvant chemotherapy. This patient was investigated, according to UK (CCLG) guidelines, with a biopsy prior to treatment and therefore avoided unnecessary chemotherapy.