Congenital Heart Diseases (CHD) include the abnormalities of the heart and great vessels that are present at birth. Individuals with CHDs may survive till adulthood. We report a case of sudden cardiac death in a young adult who was suffering from Tetralogy of Fallot (TOF) with Atrial septal defect (Pentalogy of Fallot) -a CHD. He collapsed in the hospital premises and was immediately admitted and treatment ensues, but couldn’t be saved. Autopsy revealed an oval shaped defect in interatrial septa, Right Ventricular hypertrophy, over riding of Aorta and Pulmonary atresia. Coronaries were patent. The authors aim to highlight the need of early diagnosis and timely intervention in such heart diseases. The authors also intend to add to Medical Literature that TOF may be the cause of a sudden death.

Key words: Tetralogy of Fallot, Congenital Heart Disease, Sudden Cardiac Death, Right Ventricular Dysfunction.