Orbital Rosai-Dorfman disease in a five-year-old boy

Rosai-Dorfman disease [RDD] is characterized by histiocytic proliferation and massive cervical lymphadenopathy, although some patients have extra-nodal involvement. We report a case of extranodal RDD in a five-year-old child, initially misdiagnosed as orbital inflammatory disease and treated with oral steroids. A subsequent orbital biopsy three years later confirmed the diagnosis of Rosai Dorfman disease