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We present a report of Rosai-Dorfman disease in African children, as well as highlight its familial tendency and draw attention to the different orbito/ocular presentations of this disease. An observational case review was performed. Two siblings, one with multiple subcutaneous eye lid nodules in a 12 year old female, and the younger brother who demonstrated a right fungating proptosis, participated in the review. Clinical diagnosis of Endemic Kaposi's Sarcoma and Rhabdomyosarcoma respectively were made. However, immunohistochemical examination confirmed Rosai-Dorfman disease. Patients were referred to the pediatric oncologist for systemic review and treatment. In the African setting, blindness may become a consequence of Rosai-Dorfman disease. The disease may simulate other known causes of proptosis and should be considered in differential diagnosis of orbito/ocular conditions in African children. Keywords: rosai-dorfman | familial | eyelid | african | orbit | children, Introduction Rosai-Dorfman in 1969 first described a clinicopathological entity, of Idiopathic histiocytic proliferation which was characterized by sinus histiocytosis with massive painless lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) (1,2). It [...]