Arnold-Chiari malformation is a very uncommon array of deformities in the posterior part of the cranium and hindbrain caused due to abnormal extension of the posterior brain into the spinal canal. Chiari malformation is further divided into subtypes 1, 2, and 3. The latter two are more common in pediatric forms and present at birth. The severity of symptoms depends upon the extent of herniation of the hindbrain due to herniation of the cerebellum through the foramen of the cranium. Also, there have been instances of absence of cerebellum. Multiple associated disorders like hydrocephalus due to increased intracranial pressure, then encephalocele, syrinx, or spinal deformity in the form of scoliosis have been presented in many cases. All these factors thus become a challenge to anesthesiologists for such patients. Hence evidence-based knowledge along with multidisciplinary, well-planned approach is required for its management.