Primary generalized dystonia (PGD) associated with the early-onset generalized dystonia gene (DYT1) can cause severe disability, compromising an individual's ability to perform activities of daily living. Pharmacological treatment has been inadequate in alleviating the motor dysfunctions. Deep brain stimulation of the bilateral globus pallidus internus (DBS B-GPi) has been documented to reduce these debilitating motor abnormalities. This report details the successful treatment of a DYT1-positive 13 year-old boy suffering from PGD.