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Neuroblastoma is the most common malignant disease of childhood, and it often arises from either the adrenal gland or along the sympathetic chain. The authors report a case of a 20-month-old female with a presumed primary neuroblastoma of the orbit. Radiologically, the tumor showed a brightly enhancing mass lesion of the left side with intraorbital, temporal extraorbital, transsphenoid, and intracranial components. Histopathologically, the tumor was composed of small round blue cells. Immunohistochemical staining was positive for neuron-specific enolase. To the best of the authors' knowledge, primary orbital neuroblastoma has only been previously reported once in children, although it has been reported twice in adults.