Bochdalek hernias are usually congenital and are seen with much greater frequency on the left side. Intrathoracic kidney is a very rare congenital anomaly, with only about 50 cases reported in the world literature. The incidence of intrathoracic kidney with Bochdalek hernias was reported to be less than 0.25%. A 22-month-old boy was admitted to our clinic with recurrent pulmonary infections. A chest X-ray, intravenous urogram, computed tomography, and magnetic resonance angiography revealed a right-sided Bochdalek hernia with dilated colon loops and right kidney within right hemithorax. Under a thoracotomy, a direct closure of the hernia was performed after the colon and kidney were returned to the abdomen. We report the second case, which has a diagnosis of a right Bochdalek hernia with intrathoracic kidney. Clinical correlations among this unusual combination are discussed.