Abstract
Myelomeningocele is one among the most common forms of spinal dysraphism. We report a newborn male child with leaking myelomeningocele who presented with apneic spells. He underwent a magnetic resonance imaging of the neuraxis, which revealed Chiari malformation and severe hydromyelia along with pneumocephalus. He was planned to undergo urgent repair of the neural tube defect but his parents were not willing for surgery. We report this case as spontaneous pneumocephalus secondary to open neural tube defect, which is very rare and sparsely reported.