Common variable immunodeficiency (CVID) is a primary defect of the immune system involving an increased risk of respiratory and digestive tract infections and autoimmune diseases. Recently, it has been reported that chronic inflammatory bowel disease (CIBD) might occur with increased frequency (20%) in patients with CVID. A nine-year-old boy with CVID developed CIBD during follow-up and periodic intravenous immunoglobulin administration. Serum tumor necrosis factor-a concentration, which is suggested to show disease activity in CBD, was very high. The patient's radiological evaluation, both in active and remission periods, had characteristic features of CBD. We herewith present and discuss this case with both diseases, CVID and CIBD.