Thirteen cases of intramedullary hemangioblastoma of the spinal cord are reported. The tumors were diagnosed with radiological studies, especially magnetic resonance imaging. Microsurgery was used to achieve gross total removal of the tumors in all cases. Signs and symptoms improved in 84.6% of the patients after surgery. The authors describe the diagnosis and microsurgical techniques for the excision of intramedullary hemangioblastoma of the spinal cord. This disease should be differentiated from hydromyelia, intramedullary ependymoma, and vascular malformation of the spinal cord. Total removal of the tumor is recommended.