Celiac disease is an immune-mediated disorder induced by the intake of gluten proteins involving primarily the gastrointestinal tract. Myelodysplastic syndromes are clonal hematologic disorders, expanded from myeloid stem cells of uncertain pathogenesis. Anaemia is a common finding in patients with celiac disease. Frequently it is secondary to malabsorption of iron, folic acid or vitamin B12 and usually disappears completely with a strict adherence to a gluten-free diet and supplement of deficient factors. To the best of our knowledge, in medical literature only two cases of myelodysplastic syndrome associated with celiac disease have been described. Here we describe the case of a patient affected by adult celiac disease who went into remission following a gluten free diet. Later a macrocytic anaemia without vitamin B12 or folate deficiency appeared. Hematologic tests showed findings consistent with refractory anemia with ring sideroblasts. The association of celiac disease with myelodysplastic syndromes seems not to be casual. It raises the hypothesis of a primitive immunological disorder in both diseases.