Spinal cavernous angiomas are uncommon vascular malformations in the spine accounting for 5%-12% of all spinal vascular lesions. When present in an intradural extra-medullary location, these usually present with radicular pain and neurological deficits due to mass effect (myelopathy). Herein, we present an atypical presentation of cavernous angioma in a 54-year-old man with tinnitus, headache and sensorineural hearing loss. We have also reviewed 51 cases of intradural extramedullary cavernous angiomas including our case with respect to demographic and clinical profile. A 54-year-old man presented with tinnitus in the left ear and occipital headache with neck pain and slight weakness of left-hand grip along with atrophy of thenar muscles. His pure tone audiometry (PTA) test reveled mild left sensorineural hearing loss. Magnetic resonance imaging (MRI) of cervical spine showed T2WI heterogeneously hyperintense left intradural extramedullary lesion at C7 vertebral body level. It was avidly enhancing with contrast. The patient underwent C7 laminectomy with a midline durotomy and complete excision of the lesion under neuromonitoring with sacrifice of the C8 sensory root. His symptoms improved following the surgery. The diagnosis of a cavernoma in an unusual location in the presence of cranial nerve dysfunction needs a high degree of diagnostic suspicion. Most of these cavernomas have a nerve root origin or attachment. The optimal treatment is microsurgical en bloc resection which leads to an effective resolution of both the symptoms.