Primary Immunodeficiency Diseases Presenting with Chalazia as the First Manifestation.

: Ocular manifestations in primary immunodeficiency diseases are rare, but they can be the initial manifestation. This can lead to the prompt diagnosis and treatment of the disease and achieve a reduction of severe systemic complications.: We present two cases where a recurrent giant chalazion was the symptom that led to the diagnosis and early treatment of a patient with X-linked chronic granulomatous disease (CGD), and a patient with hyperimmunoglobulin E syndrome.: Even though chalazia are common and benign, children presenting with recurrent giant chalazia or torpid evolution after surgery should be investigated for immunodeficiencies to reduce the severe and potentially fatal complications of the disease.