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Department of Neurosurgery, Seth G.S. Medical College & King Edward, Mumbai, India Spinal intradural extramedullary schwannomas commonly occur posterolateral or anterolateral to the spinal cord. A case of a pure midline ventrally situated cervicodorsal extramedullary schwannoma in a 33-year-old female patient with subacute cord compression and sphincteric involvement is presented. Spinal MR imaging showed a C2-D3 midline ventrally situated extramedullary tumor with severe spinal cord compression. It was resected through a posterolateral approach. Histology was consistent with a schwannoma. There was a gradual improvement in left-sided spasticity but the patient had mild diaphragmatic paresis. MR imaging showed no evidence of the tumor at followup after 6 months. The radiological features, pathogenesis and surgical strategies in management of these difficult tumors are discussed and the relevant literature is briefly reviewed. Keywords : Extramedullary, Intradural, Schwannoma, Spine, Surgery, Ventral location