Unilateral diffuse placoid choroidopathy can be seen in the setting of common variable immunodeficiency (CVID). A 55-year-old woman with a 20-year history of CVID presented with multiple hypopigmented placoid lesions in the right eye. Diffuse lymphadenopathy led to lymph node biopsies (mediastinal and axillary) and angiotensin-converting enzyme and serum calcium level determination, which were all normal. CVID, the most common type of significant hypogammaglobulinemia, may be associated with a placoid choroidopathy. The cause of this CVID choroidopathy is unclear at this time, but may be secondary to lymphoid hyperplasia or granulomatous disease. Copyright 2008 S. Karger AG, Basel.