Kidney is a very rare organ with teratomas and germ cell tumours. We could find only 26 reports of primary intrarenal teratomas published for 80 years. The authors present a case of Immature intrarenal Teratoma that is the first case discovered and diagnosed at National Hospital of Pediatrics. This is a 4 month-old girl who addmitted by flatulent abdomen. Physical examination and imaging findings revealed a left kidney tumour. Pathologic evaluation shows the various complements in mature (skin, brain, osteoid, cartilage...), immature (primary neuroectodermal elements) and some embryonal glomeruli and tubules. The diagnosis is Immature intrarenal Teratoma, grade III.