Celiac disease (CD) is a chronic, immune-mediated disorder, characterized by small intestinal malabsorption of nutrients after the ingestion of gluten by genetically susceptible individuals. The discovery of the wide variations in the nature and intensity of clinical presentation of CD has transformed its status, long considered a rare disease, to that of a common health problem. As patients with CD get older, they tend to present with complaints not directly referable to the gastrointestinal tract. Neurologic symptoms, caused by lesions of the central or peripheral nervous system occasionally occur in patients with CD and are poorly understood. This review focalizes on the present knowledge of the potential relationship between CD and epilepsy. The prevalence of CD among patients with epilepsy is not homogeneously distributed, probably because epilepsy encompasses a heterogeneous group of disorders. In fact, the clinical spectrum of epilepsy related to CD ranges from benign syndromes to intractable epilepsy. The precise mechanism of the potential association between CD and epilepsy is also still under discussion.