Cor biloculare, a two-chambered heart, is a rare congenital anomaly of the heart. It’s characterized by a single or common atrium communicating with a single or common ventricle by a single atrio-ventricular valve. Most patients with cor biloculare die within neonatal period or even in fetal period, and very few survivors could grow up to adult. In this article, we report an adult case with dextrocardia and cor biloculare, who survived to the 44 years of life.