Celiac disease has been associated with ataxia and other neurological signs but has not been associated with paroxysmal nonkinesigenic dyskinesias (PNKD) to date. We present a child with biopsy-proven celiac disease and a movement disorder resembling PNKD since the age of 6 months. She had complete resolution of her neurological symptoms with introduction of a gluten-free diet. Because a susceptibility locus for celiac disease has been reported on 2q33 and studies in PNKD show linkage to 2q, this report suggests further genetic investigations around this locus may be useful. We also review the literature regarding the genetic susceptibility to PNKD and celiac disease.