Cronkhite-Canada syndrome was first described in 1955. The clinical features of this rare syndrome of unknown etiology include nonhereditary gastrointestinal polyposis together with diarrhea, nail dystrophy, alopecia, and hyperpigmentation of the skin. This syndrome has been divided into five clinical types based on initial symptoms. We describe a case of Cronkhite-Canada syndrome presenting with taste disturbance as the major symptom, present a comprehensive review of the literature concerning this rare syndrome, and suggest therapeutic treatment options.