Abstract
Encephalocele is a congenital neural tube defect (NTD). The pathophysiology of the NTDs is exceedingly complex. Numerous explanations have been proposed to explain it. Double encephaloceles are highly unusual. There have only been fifteen previously reported cases of double encephalocele in the medical literature, with this index case being the oldest and first from Pakistan. A four-year-old girl presented with two occipital scalp swellings from infancy. The occipital swelling measured about 7x5x3 cm, while the suboccipital swelling measured about 7x9x5 cm. The skin over both the swellings was intact. Following a thorough history, physical examination, and radiological investigations, surgical excision and repair was performed. Postoperative recovery was uneventful. She did not develop hydrocephalus until the three month follow-up.Double encephalocele is a rare entity. The multisite closure theory appears to be the most plausible explanation for the development of multiple NTDs. The management of double encephalocele requires a case based approach.