We report on a case of nodular lymphoid hyperplasia (NLH) of the small intestine in a patient with common variable immunodeficiency (CVID) syndrome. The CVID syndrome comprises a group of heterogeneous immunological disorders. It is characterised by hypogammaglobulinemia, recurrent sinopulmonary infections, gastrointestinal disorders (including diarrhea, infestation with Giardia lamblia, chronic-atrophic gastritis and nodular lymphoid hyperplasia (NLH), and an increased risk of malignancy. NLH is frequently associated with gastrointestinal lymphomas. It has also been found in the terminal ileum of children and in adult patients with Gardner's syndrome. NLH is found in about 20% of patients with the CVID syndrome. The diagnosis of NLH requires endoscopic and bioptic-histological examinations and the determination of the immunoglobulins.