In a twenty-year series of 208 patients with anorectal malformations there were 39 infants with three or more Vater association anomalies. The neonatal mortality of Vater association patients was 28%; beyond the neonatal period only two patients died. Most early deaths were associated with the combination of cardiac anomalies and oesophageal atresia. The incidence of prematurity (30%) and lethal anomalies (15%) was high. High and complex anorectal anomalies were frequent (85%) and some kind of urogenital malformation was present in 95% of the patients. Despite the grave early prognosis and multiple hospitalisations and surgical procedures required in the management of these patients, the long-term outlook and quality of life does not differ significantly from other patients with corresponding anorectal anomalies.