Background: X-linked dystonia-parkinsonism(XDP) is a rare movement disorder primarily affecting males of Filipino descent characterized by dystonia and parkinsonism. This case illustrates a patient with a novel gene variant responsive to deep brain stimulation (DBS). Methods: Case study of Filipino male with XDP followed for 15 years. Results: A 32-year-old Filipino male presented with oromandibular and cervical dystonia which later generalized. He went on to develop parkinsonism with significant gait impairment, incomprehensible speech, and required PEG tube placement. His symptoms were refractory to pharmacologic therapy. At age 43, he underwent bilateral globus pallidus internus (GPi) DBS placement with significant improvement of his symptoms as illustrated by videos accompanying this report. He had marked improvement of gait, speech, and pharyngeal dystonia resulting in removal of his PEG tube with return to full oral intake. He continues to benefit 3 years after DBS placement. Genetic testing identified a missense hemizygous non-coding transcript exon variant TAF1 n.5776C>T which is a novel gene variant of XDP not previously reported in the literature. Conclusions: This case illustrates a patient with a novel TAF1 gene variant associated with XDP not previously reported in the literature. This variant was responsive to bilateral GPi DBS placement.