Tricuspid atresia is a congenital cardiac anomaly that is most commonly associated with normally related great vessels and less commonly with transposition of the great vessels. When tricuspid atresia is associated with dextro-transposition of the great vessels, there is a high incidence of coarctation of the aorta. This report documents the rare case of a newborn male with tricuspid atresia and normally related great arteries with both pulmonary stenosis and coarctation of the aorta.