A meningomyelocele is herniation of meninges along with neural elements through a congenital defect in the cranium or vertebral column. Reported incidence of occipital meningomyelocele is 1 in 5000 live births. Children with meningomyelocele may have varying degrees of sensory and motor deficits and other important associated congenital defects with clinical features of brain stem compression. The major anaesthetic challenges associated with myelomeningocoele repair includes difficulty in securing airway, intra-operative prone positioning, accurate assessment of blood loss and prevention of hypothermia.