Aim: Neurothekeomas are benign tumors of presumed neural sheath origin. They are primarily found in superficial soft tissues, located in the upper portion of the body. Here, we report a case of intracranial neurothekeoma. RESULTS: A 37-year-old female presented at our clinic with sudden-onset left hemifacial pain of varying duration. The physical and neurological examination findings were normal. The magnetic resonance imaging scan showed a mass compressing the pons and extending from the medial section of the left middle fossa to the posterior fossa. The patient was operated on using a left presigmoid transpetrosal approach and the mass was totally removed. ConclusIon: Neurothekeomas, also known as nerve sheath myxomas, are rare benign tumors. There have been two previous cases reported with an intracranial location. The information presented here now represents the third such case in the literature.