Introduction. The association granulomatosis combined variable immunodeficiency (CVID) is well known from the clinicians. However, the association with a large granular lymphocyte (LGL) leukemia has not been yet reported.Case report. We report a 50-year-old woman, followed for CVID associated with a granulomatous disease. During the follow-up, the patient developed a granulomatous lymphocytic interstitiel lung disease (GLILD). Secondarily, she presented a LGL leukemia.Conclusion. To our knowledge, this is the first reported case of an association between CVID and LGL leukemia. (C) 2014 Societe nationale francaise de medecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.