Objective: To present 20 cases of Pentalogy of Cantrell seen in 40 years in a single institution. Methods: We review the clinical records, image studies and pathology pieces of all cases with Pentalogy of Cantrell from 1968 to 2008. Patients were classified as: Group I (with Ectopia Cordis) and Group II (without Ectopia Cordis). Results. Twenty patients with Pentalogy of Cantrell were seen. Age at diagnosis was 2,47 months. Eleven in group I (55%). The congenital heart defects were: Group I: single ventricle: 4 (36%), double outlet of right ventricle: 3 (27%), ventricular septal defect: 2 (18%), aortic coarctation:1 (9%), atrial septal defect: 1 (9%). Group II: double outlet of right ventricle: 3 (33%), double inlet of left ventricle: 2 (22%), ventricular septal defect: 2 (22%), tetralogy of Fallot: 1 (11%), hypoplasic left ventricular syndrome: 1 (11%). Surgery was performed in twelve (60%), 5 in group I, 7 in II. Cardiac surgery was performed in 10 (50%); 5 in group I (45%), 5 in II (55%). Sixteen patients died (80%), 11 in group I (100%) and 5 in group II (55%) (p = < 0.05). Seven of 12 patients with surgery died (58%); 5/5 (100%) of group I and 2/7 (29%) of group II (p = < 0.05). Four patients are alive and asymptomatic. Conclusions: There are approximately 185 cases of Pentalogy of Cantrell reported around the world. This is the largest group of cases reported to date. Pentalogy of Cantrell has elevated mortality especially if Ectopia Cordis is associated.