Neurothekeoma is a benign nerve sheath tumor, also known as nerve sheath myxoma. It arises from the cutaneous nerves of the head and neck region. In certain cases, neurothekeoma has been reported in the breast, oral cavity, tongue, maxilla, and spinal intradural space. Intracranial neurothekeoma, however, is an extremely rare entity, with only three cases reported in the literature: one in the parasellar region, one in the deep white matter, and another one in the cerebellopontine angle. We present the case of a 40-year-old man with a very large neurothekeoma present in the posterior fossa who had no neurologic deficit on presentation.