A neonate with a congenital bronchopulmonary foregut malformation involving a left lower lobe sequestration with bronchoesophageal fistula was noted to have developmental arrest of the foregut resulting in a short esophagus, microgastria, absence of pylorus and duodenal bulb, and malrotation. Routine upper gastrointestinal imaging should be adopted for evaluation of coexisting anomalies to avoid delay in diagnosis and treatment.