An analysis of 18 infants with combined esophageal and duodenal atresia is presented. A total of 50 other associated anomalies were identified in the 18 infants. These predominantly involved the genitourinary (11), cardiac (9), anorectal (8), and gastrointestinal (8) systems. There were 6 early survivors (33% survival rate), 1 of whom died later from an unrelated cause. It is suggested that in an otherwise health infant the primary approach should be towards the esophageal atresia and tracheoesophageal fistula. A wide gastrostomy is mandatory. Repair of the duodenal obstruction may safely be postponed for a few days. In the absence of an associated tracheoesophageal fistula, a primary duodenoduodenostomy and gastrostomy with a transanastomotic feeding tube is the approach of choice.