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Anesthetic management of dystonic patients with uncontrolled involuntary movements refractory to medical management is a challenge to neuroanaesthetists. According to some studies, the prevalence of Panthothenate Kinase Associated Neurodegeneration is 1 to 9/1,000,000. Report of Deep Brain Stimulation for Hallervorden–Spatz is extremely rare in literature. “Awake” bilateral electrode placement, with microelectrode recording (MER) and stimulation with a scalp nerve block, titrated conscious sedation with Monitored Anesthesia Care (MAC) is preferable. However, in those patients needing general anesthesia, a balanced anesthesia technique with careful selection and monitored titration of anesthetic drugs ensuring MERs for precise placement and stimulation of target nuclei along with adequate plane and depth of anesthesia and prevention of awareness are essentially the key factors in the anesthetic management. Surgery is the mainstay of the disease due to poor response to medical management. Multidisciplinary collaboration and cooperation among neurologists, neurosurgeons, neuroradiologists, and neuroanaesthesiologists are imperative to ensure good patient outcomes.

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