A 40-year-old man presented to our hospital with painful tumors in his left carotid space and left knee. He had no family history of neurofibromatosis type II (NF II), no history of hearing loss or vestibular problems, and no symptoms of NF I. Magnetic resonance imaging (MRI) of the head revealed no intracranial tumors including vestibular schwannoma (VS). MRI of the left carotid and left knee demonstrated T(1)-weighted mass lesions. They were excised and all of them were pathologically diagnosed as schwannoma. Thus, this case was diagnosed as definite schwannomatosis in reference to the diagnostic criteria.