Neuromyelitis optica (NMO) is an autoimmune inflammatory disorder of the central nervous system, which predominately affects optic nerves and spinal cord. Celiac disease (CD) or gluten sensitivity is an autoimmune enteropathy triggered by ingestion of wheat gliadin and related proteins in genetically susceptible individuals. Although NMO is associated with other autoimmune disorders in around 30% of cases, an association of NMO with CD has rarely been reported. We describe two Caucasian women who, nineteen and two years after diagnosis of CD, respectively, had recurrent episodes of myelitis and optic neuritis consistent with the diagnosis of NMO. Despite numerous relapses, NMO followed an unusually mild course with no persistent neurological deficit, indicating that recurrent NMO can follow a benign course with complete remission. We discuss in detail a possible link between NMO and pre-existing CD.