Gluten-sensitive enteropathy of the Irish Setter is an immune-mediated intolerance to gluten, the protein found in wheat, barley, rye, and oats, reminiscent of human celiac disease. Intestinal histological lesions include partial villous atrophy, infiltration of the lamina propria by lymphocytes and plasma cells, and an increased intraepithelial lymphocyte count. Gluten-sensitive enteropathy is transmitted via autosomal recessive inheritance and its pathogenesis appears to involve cell-mediated immunity but not humoral immunity. In comparison to healthy dogs, levels of anti-gliadin antibodies in diseased Irish Setters are lower, although the significance of this finding is unclear. Irish Setters affected by gluten-sensitive enteropathy present with chronic intermittent diarrhea and weight loss. The use of a gluten-free diet is indispensable both for diagnosis of the disease and for therapy. In this review we discuss the similarities between gluten-sensitive enteropathy of the Irish Setter and human celiac disease.