Paroxysmal nonkinesigenic dyskinesia (PNKD) is a rare chronic disorder characterized by intermittent, non–movement-related involuntary movements. The response to currently available therapies is inconsistent and temporary. We describe here a patient with infantile-onset PNKD who failed a number of pharmaceutical agents used alone or in combination. Treatment with oxcarbazepine resulted in a substantial reduction in the frequency and severity of episodes. The patient has been followed for 4 years now, and the outcome of treatment is consistently favorable. Oxcarbazepine has been effective in managing the kinesigenic form of this disorder; however, its use has never been reported in PNKD to our knowledge. Oxcarbazepine is safer and better tolerated than most of the drugs currently used for treating PNKD, but blinded clinical trials are needed to verify its efficacy in the management of this debilitating, often difficult-to-treat disease.