Introduction Teratoid Wilms’ tumor is an uncommon variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of tumor volume [ 1 ]. To date, only 18 cases of this tumor have been published. We present a child with a left teratoid Wilms’ tumor and right dysplastic kidney, who was successfully treated with partial nephrectomy, and review all previously reported cases.