Intrathoracic kidneys are rare developmental anomalies and represent less than 5% of all congenital kidney ectopia. Ectopic intrathoracic kidneys are usually asymptomatic and have normal renal function. This disease occurs more frequently in males and on the left side. We report a case of right intrathoracic kidney with congenital complex cardiac anomalies such as single atrium, patent ductus arteriosus and tricuspid regurgitation.