Arnold-Chiari malformation is a constellation of congenital anomalies related to the hindbrain and base of the
brain. It consists of cerebellar tonsils herniation through the foramen magnum into the cervical spinal canal. Chiari
malformation is a rare entity and its etiology is not clearly known but said to be genetic. We report a case of Arnold
Chiari Malformation type 1 with holocord syrinx.
