Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by decreased serum immunoglobulin levels, and increased susceptibility to recurrent bacterial infections, malignancies, and autoimmune disorders. In this report, 2 siblings with CVID who developed Hodgkin lymphoma are presented: a 16-year-old girl at stage IIB and her 12-year-old brother at stage IIIB of Hodgkin lymphoma. Their father and 1 uncle were also affected by the same cancer with no immunodeficiency state. The presence of lymphoma should be considered in the patients with CVID, especially in those with family history of malignancies.