Hallervorden-Spatz disease (HSD) is a rare autosomal recessive disorder associated with excessive iron deposition in the basal ganglia. In general, HSD is characterized by onset in first two decade of life and by the presence of extra-pyramidal dysfunction including dystonia, rigidity, choreoathetosis. Other associated features include gait and posture disturbance, intellectual decline, seizure, tremor, dysarthria. These signs and symptoms are progressive. MRI is often demonstrated hypodensity in the basal ganglia which is probably suggestive of accumulation of iron. There is no specific treatment for HSD and 45% of patients die before reaching the age of 20 years. The managements directed at specific symptoms are often helpful. Especially, some surgical procedures like pallidotomy and gastrostomy are performed under general anesthesia. There is special need for careful management because of numerous anesthetic challenges like difficulty in cooperation, life-threatening airway obstruction and possibility of aspiration. We report a successful anesthetic management in a patient with HSD for dental procedures.