Congenital lobar emphysema (CLE) is a rare developmental disorder characterized by over distension of pulmonary lobes or segments. Often CLE presents a diagnostic and therapeutic dilemma. Here, we report a case of incidentally found CLE at fetal autopsy of terminated pregnancy due to ventriculomegaly and occipital localized encephalocele detected on sonography in the second trimester. A 41- year-old woman at gestational age of 20 weeks 4 days revealed with ventriculomegaly and occipital localized encephalocele in obstetric sonography. In fetal autopsy beside meningocele, CLE was detected. CLE is a rare malformation characterized by over-distension of pulmonary lobes. The emphysematous lungs of infants younger than six months can result from foreign body aspiration. Major differential diagnosis of hyperechogenic lung lesions include; congenital cystic adenomatoid malformation, upper airway obstruction, pulmonary sequestration, bronchogenic cysts and CLE. The exact diagnosis of CLE is based on combination of clinical, radiologic and histopathologic examinations. Antenatal remarkable findings highlight the significance of postnatal follow-up whether the lesion echogenicity differs in size, even gets disappeared in the prenatal course. In English literature, this case is the first CLE associated with meningocele.