Destombes Rosai Dorfman disease and sinus histocytosis with massive lymphadenopathy is a rare benign idiopathic proliferative disease of phagocytic histiocytes. Approximately 80% of patient present with painless massive cervical lymphadenopathy. It rarely involves other sites for example CNS, eyes, upper respiratory tract and skin. A case of Destombes Rosai Dorfman disease is reported with history of gradual protrusion of left eye since many years. The histological features were consistent with the diagnosis
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