Meningeal melanocytoma is a rare tumor of nervous system, which originates from leptomeningeal melanocytes.The locations of melanocytoma in the nervous system are most frequently in the posterior fossa or along the spinal cord, and usually appear as an extra-axial mass.The manifestations of tumor are most often due to its compressing effect on adjacent nervous structures that causes various neurological signs and symptoms depending on its locations. It may also cause superficial siderosis of the central nervous system [1].In this case we describe another manifestation of this tumor which raised intracranial pressure and developed its neurological signs and symptoms. The patient was a 33-year old man with a two-year history of headache and tinnitus, transient diplopia, and had also a three- month history of progressive bilateral visual and hearing loss. The medical investigations of the patient reveal raised intracranial pressure (RICP) with a high concentration of protein in the cerebrospinal fluid, and an extra-axial mass at the T11-12 level in magnetic resonance imaging of the spinal cord. The patient underwent surgical removal of the tumor, in which the pathological study characterized the tumor as a meningeal melanocytoma. After surgery the CSF pressure returnedto normal state, and its protein level decreased. The patient's hearing loss improved significantly but the visual defect did not change.Base on various causes of the RICP,especially when there is abnormality in CSF protein without any known cause,we must consider melanocytoma as a treatable cause, and thus in such patients, performing spinal cord magnetic resonance imaging (MRI) is a valuble technique for diagnosis as well as investigation.