Esophageal lung is an extremely rare type of bronchopulmonary foregut malformation in which the main stem bronchus is anomalously connected to the esophagus instead of the trachea and is supplied by branches of pulmonary artery. Less than 25 cases have been reported so far in literature. We hereby report first case of esophageal lung associated with VACTREL anomaly. An 8-month-old female child with weight of 4.7 kg presented with recurrent lower respiratory infections. She was diagnosed to have esophageal lung on basis of clinical examination, chest X-ray f/b computed tomogram of chest and esophagobronchoscopy. There was a bifid vertebra at D9 level with scoliosis, an ectopic left kidney and dextrocardia with patent foramen ovale. Thus, this case was classified as having VACTREL association. Patient was successfully operated for excision of the hypoplastic lung via thoracotomy. She recovered well and gaining weight at 3 months f/u without any respiratory infection since surgery. High index of suspicion is necessary for the diagnosis of esophageal lung when common causes of recurrent respiratory infections are ruled out.