Abstract： Common variable immune deficiency (CVID) is one of the most common primary immunodeficiency diseases, which is characterized by decreased serum immunoglobulin levels and B lymphocyte dysfunction. CVID occurs in both children and adults. Lymphocytic interstitial pneumonia (LIP) is a rare interstitial lung disease characterized by massive infiltration of lymphocytes in the pulmonary interstitia. It is closely related to a variety of systemic diseases, such as AIDS, Epstein-Barr virus infection, autoimmune diseases and various immunodeficiency states, including CVID. There are few data on the pathogenesis, diagnosis and treatments of these two diseases in the previous literatures. Here, we reported one case of CVID with LIP in our hospital is reported as follows. The patient had repeated infection, hepatosplenomegaly, decreased IgG, IgA and IgM, and no other causes of hypogammaglobulinemia were found. Combined with the results of gene detection and lung biopsy, the patient was finally diagnosed as CVID with LIP. According to the initial immunoglobulin results of hematological examination, immunoglobulin treatment was administrated for half a year, and no symptoms of infection occurred again. Up till now the outpatient follow-up has remained stable.