Background: An intracranial lesion of intravascular papillary endothelial hyperplasia (IPEH) is extremely rare and often difficult to resect completely. We describe a case in which a lesion at the superior orbital fissure was successfully treated by postoperative radiosurgery. Case Description: A 41-year-old woman presented with progressive left abducens nerve paresis and left facial sensory disturbance over a 1-year period. Radiological examination demonstrated a left middle fossa tumor involved in the cavernous sinus. About a half removal of the tumor was performed. The pathological diagnosis was IPEH. The residual tumor was treated with gamma knife radiosurgery. Conclusion: Gamma knife radiosurgery was effective to reduce the residual tumor in the present case. During a follow-up period of 2 years, the lesion has not recurred. This report suggests that additional gamma knife radiosurgery after incomplete resection of the lesion could be an alternative therapeutic modality. (c) 2005 Elsevier Inc. All rights reserved.