Congenital diaphragmatic hernia is a rare congenital malformation, as well as kidney ectopia. Among kidney ectopias, the intrathoracic one is the rarest. Those malformations concern more frequently boys, and affected more the left than the right side. Their association is poorly reported in the literature. We report the rare case of an early sonographic prenatal diagnosis of intrathoracic kidney at 22 weeks of gestation in a female fetus, completed on the follow-up by the diagnosis of an associated diaphragmatic hernia at 33 weeks of gestation. If chest mass is diagnosed prenatally or in neonate, ITK should be considered in a differential diagnosis, all the more if the ipsilateral renal fossa is empty. An associated DH should be searched if ITK is confirmed. Isolated ITK usually requires no specific treatment, in contrast with ITK associated with DH.

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