Common variable immunodeficiency (CVID) is an idiopathic state of immune dysregulation that results in impaired antibody synthesis and the development of recurrent bacterial infections. CVID patients have been known to have an increased tendency to develop autoimmune manifestations. A ten-year-old girl who had been followed as juvenile rheumatoid arthritis JRA) since 3-years-old diagnosed CVID is presented. She had recurrent upper respiratory tract infections, splenomegaly and oligoarthritis. Immunologic studies revealed that hypogammaglobulinemia, reversed CD4/CD8 ratio and deficient antibody response to tetanus antigen. CVID was diagnosed and intravenous immunoglobulin treatment (400 mg/kg every 3 weeks) was started. After two months of the therapy, she did not have any complaints and clinical examination was normal except splenomegaly.