Abstract
Arnold Chiari malformation (ACM) is defined as protrusion of the meninges and brain components into a congenital defect in the cranium or in the vertebral column. It was originally described by Austrian pathologist Hans Chiari. Among the four types, type-III ACM is the rarest and may associate with encephalocele. We report a case of type-III ACM associated with large occipitomeningoencephalocele with herniation of dysmorphic cerebellum, vermis, kinking/herniation of medulla with cerebrospinal fluid, tethering of spinal cord with posterior arch defect of C1–C3 vertebrae. The anaesthetic challenge for such patients lies in the proper preoperative work up; proper positioning of the patient during intubation; safe anaesthetic induction; intraoperative management of intracranial pressure, normothermia, and fluid and blood loss; and postoperative planning of extubation to prevent aspiration are the prerequisites to overcome the dificult airway management and anaesthetic challenge in the management of type III ACM.