Giant extralobar pulmonary sequestration in newborns is still relatively rare in pulmonary diseases, and there are few relevant studies published. A neonate with the giant extralobar pulmonary sequestration accompanied by severe pleural effusion was reported here. After 12 days of birth, the diseased lung tissue was surgically extracted. The patient had an uneventful postoperative recovery and was discharged from the hospital. The case shows the advantage of early surgical treatment to extralobar pulmonary sequestration with severe pleural effusion in neonatal period.