Peripheral neuropathy (PN) is one of the most frequently reported neurologic manifestations associated with celiac disease (CD), a multigenetic, T-cell-mediated autoimmune disorder that results from a loss of tolerance to gluten. Sensory axonal and small fiber sensory polyneuropathies are the most frequently reported PN subtypes. Multifocal motor or sensorimotor neuropathies and a more fulminant neuropathy, associated with ataxia and other neurologic manifestations, also have been reported. The effect of a gluten-free diet on CD-associated PN has not been studied systematically or prospectively; nevertheless, a gluten-free diet currently is the cornerstone of therapy. Although idiopathic ataxia associated with anti-gliadin antibodies and other neurologic complications have been reported to respond to this diet; there is data that indicate that neurologic manifestations may develop or persist, independent of gluten exposure. There is evidence to suggest that inflammatory processes may be involved. Immunomodulatory agents (such as intravenous immunoglobulin or infliximab), described to be beneficial in the treatment of refractory CD or CD-associated ataxia, may have a role in the management of CD-associated PN.