Esophageal lung is a rare entity that results from embryological alterations during the formation of the ventral wall of the anterior intestine. The clinical manifestations of this pathology are vague, including respiratory or digestive symptoms, repetitive respiratory infections, dysphagia, or inability to swallow. The management is based on the exact anatomical and vascular abnormalities. We report the diagnostic and therapeutic approach in a preterm boy with esophageal lung. Also, we present a three-dimensional model for the classification of this pathology. In conclusion, the management relies on proper definition of the anatomy and the surgical strategy.