ObjectiveTo investigate the pathogenesis, clinical manifestations, diagnosis and treatment of common variable immune deficiency (CVID).MethodsOne case of CVID with cellular immunodeficiency leading to bronchiectasis and liver cirrhosis was analyzed retrospectively. Relevant literatures were also searched through WanFang Database, China National Knowledge Infrastructure and PubMed, Ovid, Embase, Cochrane using the key words " common variable immunodeficiency”, " common variable hypogammaglobulinemia” in Chinese and English.ResultsA 52-year-old female patient, complained of cough, expectoration for 20 years, edema for 7 years and aggravated for 3 months with a history of recurrent respiratory infections was hospitalized in the West China Hospital of Sichuan University. The chest computed tomography revealed bronchiectasis, liver cirrhosis and portal hypertension. Laboratory tests showed remarkable hypogammaglobulinemia. The CD4+ T-cell count was below the normal range. Probable diagnosis of CVID was made based on clinical characteristics and laboratory tests. Immunoglobulin infusion with a dose of 20 g was given and the symptoms were relieved. About 288 case reports including 8 000 patients were searched. Most of them were reported individually. Conclusions CVID has a low morbidity and is rare in China. It is mostly caused by genetic factors. When there are recurrent infections in common areas of body, infections in rare areas or infections of conditioned pathogen, clinicians should be vigilant and give intervention as soon as possible. Family and genetic researches could be done when permitted.