Introduction: Neurofibromatosis type I (NF-1) is a common inherited, multisystem, neurocutaneous disorder that predisposes to the development of benign and malignant tumors. Orbital-temporal manifestations of neurofibromatosis are not common. Case Report: We report a rare case of atypical presentation of NF-1 which presented to us with incidental asymptomatic unilateral pulsatile eye. After investigation, the patient was found to have unilateral partial sphenoid bone defect. No other orbital-temporal manifestation of neurofibromatosis was noted. Conclusion: The pathogenesis of unilateral greater wing defect in NF-1 is still unclear. Some authors have reported that the sphenoid bone defect may progress. If the defect does progress, we need to follow-up to monitor sphenoid bone defect manifestation in every orbital-temporal NF-1 patient.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.