We describe the clinical course of 120 children with tricuspid atresia (TA) attended in the Hospital de Cardiología y Neumología, "Dr. Luis Méndez", del Centro Médico Nacional. There were 61 males and 59 females. The age of presentation was in 79 newborn babies, seventeen between one and six months old, 20 between six and 24 months old, and four with two or more years old. The clinical picture was hypoxic spells in 89% and congestive heart failure in the others. The chest film showed cardiomegaly in 85%, with diminished pulmonary flow in 48%, increased flow in 27.5%, and normal in 9%. The electrocardiogram with superior left axis deviation in 94%, right atrial hypertrophy in 58%, left atrial hypertrophy in 47.5% and left ventricular hypertrophy in 96%. TA was classified as type I in 103 children, type Ic in 70, Ib in 27 and Ia in six, and type II in seventeen children, with eight IIc, six IIb and three IIa. In 44 the management was medical, 63 underwent systemic-pulmonary anastomosis, 37 of them with Blalock-Taussig shunt and Fontan procedure in thirteen children. There were 21 deaths. This survey is compared with the literature and from this point we make management recommendations.