A large subtorcular occipital encephalocele in neonate carries higher risk of associated hindbrain anomalies and
secondary process for neurological deterioration which predict the surgical outcomes and long-term prognosis. The dysfunction of the lower cranial nerves often leads to worsening of neurological status from poor respiratory function and repeated aspiration pneumonia. The aims of repairing encephalocele include a good closure of the defect, preservation or restoration of neurological functions and better cosmetic results. The author presented a successful surgical strategy for management of a 4-month-old infant with a large subtorcular occipital encephalocele presented with bilateral vocal cord paralysis and swallowing dysfunction. A step by step approach unlocked the main mechanism(s) of reversible lower cranial dysfunctions in
this specific situation, including the increased intracranial pressure and shifting of the axis of lower brain stem.

Keywords: Occipital encephalocele, Vocal cord paralysis, Lower cranial nerves dysfunction