Between 1958 and 1983, 92 neonates with omphalocele were admitted to Ste-Justine Hospital. The male to female ratio was 3:2. Birth weight ranged from 1,450 to 5,100 g (mean 2,786 g). Associated anomalies, apart from malrotation, were present in 45%. They were cardiovascular (18.4%), vesico-intestinal fissure or bladder exstrophy (11.9%), Beckwith Wiedeman (6.5%), and chromosomal abnormalities (7.5%). Seven patients were not treated. Topical applications were used in seven cases (1 survivor). In 65%, primary closure was achieved; the mortality rate was 20% with a mean hospital stay of 17.5 days. Silastic was used in 12 cases with a mortality of 5/12 and a mean hospital stay of 82.1 days. The mean number of reductions was 6.7. Omphalocele rupture did not influence mortality. Prior to 1974 the mortality rate was 50%. Since 1974 it has decreased to 31.5%. The advent of total parenteral nutrition (TPN) was an important factor in decreasing the mortality. Prior to 1974, 23 patients survived, only one with a major associated anomaly (Fallot). After 1973, 35 survived, 13 having a major associated anomaly. Overall mortality was 9% when no other major malformations were present. A retrospective study of 92 cases of omphalocele over a 25-year period reveals an overall mortality rate of 37%. Death was associated almost exclusively with additional congenital anomalies. Birthweight in itself was not a determining prognostic factor. With the advent of TPN and a better knowledge of the mechanical ventilation of the neonate the results are better and involve the survival of a greater number of patients with serious associated malformations.