In celiac disease, the ingestion of gluten-containing cereals, such as wheat, rye, and barley, results in small-bowel mucosal inflammation and villous atrophy with crypt hyperplasia. The prevalence of the condition may be as high as 1% in the adult population. The disease can also embrace various extraintestinal manifestations, of which dermatitis herpetiformis is the best known. Earlier, dermatitis herpetiformis was considered a skin disease occurring often concomitantly with celiac disease. At present, a body of evidence shows that dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and affects approximately 25% of patients with celiac disease. Both conditions can appear in the same family and are closely linked to HLA class II locus in chromosome 6; 90% of patients have HLA DQ2 and, almost all the remainder, HLA DQ8. All patients with dermatitis herpetiformis have at least some-degree of mucosal inflammation or lesion consistent with celiac disease. The etiology of celiac disease in not fully understood, but tissue transglutaminase seems to be the predominant autoantigen both in the intestine and the skin. Serum antibodies against tissue transglutaminase can be used in the serologic screening and follow-up of dietary compliance of patients with celiac disease. Gluten-free diet is essential in the treatment of both conditions, and oral dapsone is usually needed in newly detected dermatitis herpetiformis in order to alleviate symptoms. Oral mucosal lesions, alopecia areata, and vitiligo probably occur more frequently in patients with dermatitis herpetiformis than in the general population. By contrast, the reported association of celiac disease with psoriasis seems to be coincidental.