Background: Obstructive gastrointestinal (GI) malformations are one of the most important congenital problems and have different clinical manifestations, which depend on the severity and location. Although, complete obstructive lesions cause classic intestinal obstructive symptoms in infants, incomplete obstruction might be asymptomatic until adulthood and sometimes cause unusual symptoms, which need extensive work up. Moreover, this delay could produce significant complications. Near all of these lesions are surgically correctable; therefore awareness of this possibility is important in order to prompt appropriate management and referral plan.

Case report: In this report, we introduce five infants who had congenital GI malformations with different presentations such as vomiting, hematemesis, growth failure and steatorrhea. Appropriate time of diagnosis caused surgical correction of lesions except in one patient with very unusual presentation who was diagnosed late. As a result, in this case, severe growth and developmental delay appeared.

Conclusion: Congenital gastrointestinal obstruction has different manifestations. Awareness can help to survive patients.