The thoracic cavity is the rarest location of all the renal ectopic sites. We report a rare case of an acquired thoracic kidney associated with a previous congenital diaphragmatic hernia in a child with nephrotic syndrome. Although only 13 cases of the thoracic kidney in the pediatric age group have been described in worldwide reports during the past 25 years, we present the first to be associated with a recurrent diaphragmatic hernia. The classification, differential diagnosis, and
management options of this rare form of ectopia are discussed.