The co-occurrence of embryonal tumors originating in the kidney and the brain are very rare. A 2 months old girl presented with a significantly increased head circumference. She received tumor removal with insertion of a ventriculo-peritoneal shunt after brain MRI showed brain tumor with hydrocephalus. Her tumor was pinealoblastoma. She did not receive any treatments after surgery. We evaluated abdominal computerized tomography for tumor dissemination because of the shunt. We incidently found a tumor mass on the right kidney, and performed total resection of renal mass. The renal mass was Wilms tumor. She died within 2 months after chemotherapy. We report a case of pinealoblastoma with a consequently discovered Wilms tumor.