We present a case report and review of the literature that illustrates many key features of patients with common variable immunodeficiency (CVID). These patients frequently present with repeated infections with a variety of different microorganisms. Recurrent sinopulmonary infections can lead to serious chronic complications such as bronchiectasis, and gastrointestinal infections can result in malabsorption. In addition to serious infection, CVID is associated with a number of comorbid disorders including a variety of autoimmune diseases and neoplasms. Here, we provide an illustrative case report and discuss the primary features and therapy for patients with CVID.