Diagnosis of intra-cochlear pathology has become easier with contribution of the development of MR technology and increased use of MR imaging in the evaluation of sensorineural hearing loss. Intracochlear schwannoma (ICS) is a rare tumor which is originated from schwann cell located in spiral ganglion. Its predominant symptom is progressive sensorineural hearing loss. Small size, location, slow growth rate and variable clinical features of this tumor make correct diagnosis difficult and are often delayed for years. Clinical history, physical examination, imaging features, and treatment options of this rare disease are discussed.