Encephalocele is a mesodermal defect in the skull bones and duramater. Parietal encephalocele is a rare congenital anomaly of newborn with variable prognostic value. The authors report a case of a very large inter parietal encephalocele with no associated other system malformations. A midline inter parietal encephalocele is much rare, earlier reported cases were posterior parietal in location. Such cases can be successfully operated upon with a very good outcome. A unique case of a 18 day neonate, with swelling over scalp was evaluated by the neurosurgical team and the patient underwent neurosurgery. In planning the strategy for management of encephalocele, one needs to take into consideration the site, size, contents, patency of CSF pathway, neurological status and other associated anomalies. Inspite of such a big encephalocele in an atypical location, excision and repair gave excellent results.
Keywords: Congenital anomaly; Parietal encephalocele; Vertex encephalocele.