Drainage of the inferior vena cava to the left atrium is an extremely unusual congenital heart disease. We describe a 54-year-old woman, in whom the diagnosis was suggested by transthoracic echocardiography, and then confirmed by a transesophageal exam and magnetic resonance imaging, which also revealed an associated secundum atrial septal defect. Surgical management involved reconstruction of the interatrial septum to include the inferior vena cava in the right atrium. The few previously reported cases in the literature are reviewed.