This paper describes the perinatal and postnatal outcome of a case considered to have a fetal intra-abdominal extralobar pulmonary sequestration.Routine midtrimester fetal ultrasonographic scan detected an abdominal echogenic mass between aorta and stomach. It was considered primarily as an intra-abdominal extralobar pulmonary sequestration. At postnatal 2 months, computed tomography scan with intravenous contrast described it as an extralobar pulmonary sequestration at the level of diaphragmatic hiatus by showing the aberrant blood supply to the sequestration with venous drainage into the portal confluence. The patient is now 4 years old and asymptomatic; she is being followed-up by a pediatric surgeon.Intra-abdominal extralobar pulmonary sequestrations usually remain asymptomatic throughout life; they may be safely observed without surgery unless they become symptomatic or show any change in the characteristics of the radiologic appearance.