Pulmonary agenesis is often associated with anomalies in the cardiovascular, gastrointestinal, genitourinary, or musculoskeletal systems.1,2–4,5 Both lungs are equally affected but right lung agenesis shows a worse prognosis.3,5 Here we report a similar case of congenital pulmonary agenesis with dextrocardia, term born live born female baby who survived for 30 days.