Rosai-Dorfman disease manifesting as an epibulbar ocular tumour

The authors report a rare case of extranodal Rosai-Dorfman disease without systemic involvement. A 14-year-old girl presented with a slowly progressive, non-tender, well-circumscribed, perilimbal nodule in the right eye. Excisional biopsy was performed and histopathologic and immunohistochemical analysis established the diagnosis. Systemic evaluation revealed no other masses. After complete excision of the epibulbar lesion, the patient is asymptomatic and remains on regular follow up with no signs of recurrence. Rosai-Dorfman disease is an infrequent, benign histiocytic disorder that can rarely manifest as an exclusive extranodal mass. We report a case of solitary epibulbar Rosai-Dorfman disease, successfully treated with complete surgical excision of the lesion.