Congenital intrathoracic kidney with right Bochdalek defect

Intrathoracic kidney is a rare congenital anomaly. Since most reported cases are asymptomatic, it is extremely rare for this ectopia to be diagnosed in the neonatal period. We report a male infant with right intrathoracic kidney associated with Bochdalek defect. Chest X-ray demonstrated a right posterior mediastinal mass and intestinal gas in the right lung field. Contrast-enhanced CT and intravenous urography led to a diagnosis of intrathoracic kidney. Due to the presence of Bochdalek defect, the intrathoracic kidney was reduced into the abdominal cavity at the time of diaphragmatic repair. The intrathoracic kidney with attached adrenal gland was located at the level of the carina and was covered with protruded retroperitoneum. The kidney was thought to have been pushed this high by the small intestine and left lobe of the liver, which had also herniated through the defect. Postoperative hemodynamics and renal function were normal.