10–28% of the patients with chronic coeliac disease develop a second malignancy, such as non-Hodgkin’s lymphomas, particularly of the histiocytic type, and adenocarcinomas. The second tumor may arise in the intestinal tract or in other organs. We describe here a patient who developed a tartrate-resistant, acid phosphatase-positive, hairy-cell leukemia after a history of chronic coeliac disease. In the literature, no similar case has been described as yet.