Common variable immunodeficiency (CVID) is a disease characterized by severe antibody deficiency due to impaired B cell differentiation. It represents the most common form of primary immunodeficiency in children and adults, and its clinical manifestations include recurrent infections and chronic lung disease, gastrointestinal infections, and autoimmunity. Here, we present the case of a 47-year-old female patient with a history of CVID and recurrent Campylobacter jejuni bacteremia. She was undergoing biweekly administration of intravenous immunoglobulin for over 15 years. During hospitalization rapidly progressive oliguric renal failure was observed in association with anasarca and nephrotic syndrome. Bilateral nephromegaly was noted on an abdominal pelvic computed tomography scan. Renal biopsy was consistent with amyloidosis, and serum amyloid A protein was elevated. The diagnosis of AA amyloidosis secondary to CVID was made. The patient was started on hemodialysis and weekly intravenous immunoglobulin administration with favorable clinical outcomes.