Purpose: To demonstrate the clinical, radiographic, intraoperative and histopathologic presentation of a localized neurofibroma of the orbit. Methods: Clinicopathologic case report. Results: A 25-year-old man was examined for inferior displacement of the right globe. Physical examination and neuro-imaging identified a localized solid tissue mass of the superior orbit. Histopathologic evaluation after complete surgical resection proved the lesion to be a localized neurofibroma. Conclusions: The patient's history, clinical presentation, and imaging studies were strongly characteristic for this infrequently encountered orbital lesion. Several imaging features, including multilobulation, multiplicity, ring-configured contrast enhancement, and magnetic resonance imaging (MRI) signal intensity heterogeneity, when present in combination, may be reasonably diagnostic of this tumor.
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