Neurothekeoma is a benign cutaneous nerve-sheath tumor. The authors describe the case of a 16-year-old girl with a parasellar neurothekeoma that was successfully removed. Magnetic resonance imaging with gadolinium administration revealed bright enhancement of the peripheral portions and nonhomogeneous uptake in the central areas, which corresponded histologically to the vascular capsule and the myxoid center. It was concluded that: 1) intracranial neurothekeoma does exist, and 2) surgical resection appears to be the definitive therapy.