We present the case of a 20-year-old male with intermittent right upper extremity numbness for 3 months. His pain perception and temperature sensation were severely disturbed. An incidental magnetic resonance imaging (MRI) finding of one small intramedullary enhancing nodule at spinal cord level T10-11 with long-segment syrinx formation suggested the diagnosis of spinal hemangioblastoma with syringomyelia. Surgical removal of the tumor and decompression of the spinal cord with opening of the syrinx were performed smoothly, and the pathology confirmed the diagnosis of spinal hemangioblastoma. Reviewing the literature, MRI is the examination of choice for spinal hemangioblastomas, and is helpful in preoperative planning and the differential diagnosis of spinal cord neoplasms and vascular lesions.