Celiac disease (CD) is an autoimmune gluten-dependent enteropathy characterized by atrophy of the intestinal villi that improves after a gluten-free diet. CD is often associated with extraintestinal manifestations. In the past few years, growing evidence has documented the involvement of skin diseases among the extraintestinal manifestations of CD. This association could be related to the impairment of intestinal absorption and motility, other than to immunological and hormonal changes. The aim of this review is to report all CD-associated skin manifestations described in the literature and to analyze the possible mechanisms involved in this association. The opportunity to evaluate the possible presence of CD in patients affected by skin disorders is discussed.