Double-chambered right ventricle (DCRV) is a rare congenital heart disease in which the right ventricle (RV) is hypertrophied due to anomalous muscle bundles. Anomalous left coronary artery from pulmonary artery (ALCAPA) is also a rare lesion constituting 1 in 3,00,000 live births. Isolated LAD from pulmonary artery (ALADAPA) is further rare. We report a case of DCRV associated with Anomalous left anterior descending Artery from Pulmonary artery (ALADAPA). The patient was initially diagnosed to have DCRV and on further evaluation associated anomalous left anterior descending artery from pulmonary artery was detected. The patient underwent DCRV repair and Takauchi repair and had an uneventful post operative recovery.

Keywords: Congenital Heart Disease; Double Chamber Right Ventricle; DCRV; ALCAPA; ALADAPA.