Introduction: Common Variable Immunodeficiency (CVID) is one of the primary immunodeficiencies that its patients can develop its symptoms since infancy till senility; however, it usually manifests between the age of 15 and 40 years. It is characterized by low antibody levels and recurrent infections.  Individuals with CVID are more prone to autoimmune diseases and malignancy.
Case presentation: We presented a 17-year-old girl with a documented CVID, who was treated with intravenous immunoglobulin and prophylactic antibiotics.  She suffered from CVID from the age of 13 to the study time. She was hospitalized due to illness, fever, and severe dyspnea. Her chest X-ray revealed cardiomegaly; thus, high-resolution thorax CT scan and echocardiography were performed which revealed pericardial effusion. No underlying autoimmune diseases were detected in our assessments. Significant clinical, radiographic and physiological improvements were achieved after conducting an appropriate therapy. The patient was followed for two years and showed no clinical and laboratory findings in respect to autoimmune diseases. In our case, the pericardial effusion was present without any evidence of autoimmunity.
Conclusions: Cardiac manifestation and complications are rare in CVID patients in the absence of an autoimmune disease; however, they should be considered.