Pure ventral midline giant schwannoma is an extremely rare entity. Spinal intradural extramedullary schwannomas commonly occur posterolateral or anterolateral to the spinal cord. A case of a pure midline ventrally situated giant pan cervical extramedullary schwannoma in an 18-year-old male patient with compressive myelopathy and sphincter involvement is presented. Spinal MR imaging showed a midline ventrally situated extramedullary tumor with severe spinal cord compression extending from clivus to C7 vertebra. It was resected through a posterolateral approach. Histology was consistent with a schwannoma. Post operative MR imaging showed no evidence of the tumor. The radiological features, pathogenesis and surgical strategies in management of these difficult tumors are discussed and the relevant literature is briefly reviewed.