Teratomas are neoplasms that arise from pluripotent cells and can differentiate along one or more embryonic germ lines. Renal teratoma is an exceedingly rare condition. Teratomas commonly arise in the gonads, sacrococcygeal region, pineal gland, and retroperitoneum. They present mainly as an abdominal mass with few other symptoms. Majority of the tumors are benign, situated on the left side and para renal, occasional lesions are bilateral. If diagnosed early, they are amenable to curative excision.Renal teratomas are rare and most have been dismissed as cases of teratoid nephroblastomas or retroperitoneal teratomas secondarily invading the kidney. The differentiation between these two neoplasms in the kidney is often problematic.We present a case of intrarenal immature teratoma in a six-month-old baby girl.Virtual slidesThe virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1746249869599954.