Common variable immunodeficiency (CVID) is a primary defect that is characterized by impaired antibody production. CVID patients may develop recurrent infections, autoimmune disorders and/or systemic granulomatosis. It is well documented that CVID patients are at risk to develop malignant lymphomas. However, to the best of our knowledge, lymphoma complicating the course of CVID associated with systemic granulomatosis has never been reported. We describe two CVID patients with systemic granulomatosis who developed B-cell lymphomas, one related to Epstein Barr virus infection, 5 and 12 yr after CVID had been diagnosed.