Abstract—Congenital lung anomalies are categorized as pulmonary agenesis, aplasia and hypoplasia with distinct clinical implications. A baby came with repeated infection of thorax. A detailed evaluation including computed tomography of the thorax and fiberoptic bronchoscopy led to a diagnosis of left lung aplasia. He also had wheezing dyspnea, which was confirmed as bronchial asthma. Congenital lung defects with associated asthma was reported only twice till date. A high index of suspicion is required to recognize such a patient.