Abstract
A 10-year-old girl presented to us with inferonasally displaced, non-axial proptosis of oculus dexter (OD), with ipsilateral, painless massive cervical lymphadenopathy of ten weeks duration. After a detailed hematological work-up and imaging, the histological evidence obtained on cervical lymph node biopsy established the diagnosis of Rosai-Dorfman disease. Although, Rosai-Dorfman disease of unilateral lacrimal gland is extremely infrequent, a high degree of suspicion is warranted in a case of young patient presenting with unilateral isolated lacrimal gland swelling and associated lymphadenopathy, wherein no other cause is found. Treatment protocol should be individualized as per the extent of systemic involvement and functional disability.