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Sinus histiocytosis with massive lymphadenopathy (also known as Rosai Dorfman syndrome) is a unique disease of unknown etiology with a childhood predilection. It is an uncommon benign condition, often confused with lymphoma. Patients usually present with massive, painless, bilateral cervical lymphadenopathy and clinical course is variable. This condition has been sporadically reported in Indian literature. Here we report a 26-year-old women presenting with a painless bilateral cervical lymphadenopathy. A FNAC from cervical lymph node was compatible with Rosai Dorfman syndrome and confirmed histopathologically.

Rosai Dorfman syndrome — A rare clinical entity