A series of 100 patients with gastroschisis and omphalocele is presented. It is emphasized that gastroschisis generally has a strikingly homogeneous clinical presentation, while the omphalocele has a more heterogeneous clinical presentation, varying from the smallest to the largest, nearly incorrectable type. The main problem in the two groups is the problem of the closure of the defect. In gastroschisis there are, in addition, two problems related to the length of the intestinal tract and the tendency to peritonitis and septicaemia. In omphalocele, however, the major problem is the associated anomalies. The experience from the present material indicates that greater efforts must be used in the future in trying to avoid hypothermia, both during transportation and during operation. Furthermore, greater efforts must be made in closing the abdominal wall defect primarily in both groups of patients. More liberal use of respirator and total parenteral nutrition, mainly by peripheral veins, has obviously contributed to the improvement in our results from 1 60% mortality rate in the first nine-year period to a 37% mortality rate in the last nine-year period in the omphalocele group. Our best results have been obtained during the last 3 years with 2 deaths in the last 12 patients who all were treated by primary closure. In the gastroschisis group a survival rate of 69% in the whole series and of 74% in those who were primarily completely closed must be looked upon as progress in the light of earlier reports of survival rates of 36% to 43%.