A case of subglottic narrowing caused by an extranodal deposit of sinus histiocytosis in a 29-year-old man is reported. The characteristic features of Rosai-Dorfman disease, such as massive painless cervical lymphadenopathy, fever, raised erythrocyte sedimentation rate and raised white cell count, were all found to be absent. However, typical histological features, such as diffuse lymphoplasmacytic infiltrate, Russel bodies, foamy histiocytes and histiocytes with emperipoiesis, confirmed the diagnosis. Rosai-Dorfman disease is an uncommon disorder and its presentation with progressive subglottic narrowing is extremely rare. Our experience in the management of this condition is reported and the review of literature is discussed.
