We review 3 pediatric cases of extramedullary hematopoietic disease occurring in the orbit, ethmoid sinuses, and parotid gland, and present a review of the literature. Each of the 3 patients was taken to the operating room, and the biopsy specimens obtained were successful in establishing the diagnosis in every case. Head and neck manifestations of extramedullary hematopoietic disease are rare, indeed. However, these cases demonstrate that it is important for otolaryngologists, pediatricians, primary-care physicians, radiologists, and pathologists to maintain a high index of suspicion for extramedullary presentations of hematopoietic disease in the head and neck. A coordinated multidisciplinary approach, including the appropriate surgical approach for biopsy, will facilitate determination of the diagnosis and treatment plan.

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