Introduction: Neurofibromatosis-II is a genetically inherited condition characterized by the development of several central nervous system tumors, the most common of which are vestibular schwannomas with or without peripheral symptoms such as cataracts or cutaneous neurofibromas. NF2, unlike its type 1 sibling, is a rare condition. We present a classic neurofibromatosis type 2 with a wide range of clinical signs and typical neuroimaging findings. Information about the patient: In tertiary rural hospital, a male child was admitted with a chief complaint of severe headache since one month, difficulty in sitting, the problem with balancing, ringing in the ear, backache etc. Main symptoms and important clinical findings: In tertiary rural hospital, a male child was admitted with a chief complaint of severe headache since one month, difficulty in sitting, problem with balancing, ringing sensation in the ear, backache etc. Patients underwent various investigations such as complete history taking, physical examination, complete blood count, CT scan, MRI, X-Ray, and lumbar puncture all investigations are done. Conclusion: Neurofibromatosis type 2 with pseudo meningocele is caused because of the mutation in the gene for a protein that regulate a growth of nerve tissue. After getting treatment as per doctors prescribed patient symptoms has minimize.