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OBJECTIVE Syringomyelia was predominantly caused by Chiari malformation or intramedullary ependymoma. The goal of this study was to identify factors related to clinical outcomes and spinal hemangioblastoma (SH)-induced syringomyelia formation in a single series of patients. PATIENT AND METHODS Thirty-eight patients with SH were treated with microsurgery from January 2013 to December 2018. Clinical features and related factors were retrospectively analyzed in SH patients with and without syringomyelia. RESULTS Out of the total number of SH patients, 21 presented with remarkable syringomyelia, resulting in an incidence of 55.26% (21/38).Gross total resection was achieved in 36 cases (94.73%), and subtotal resection was obtained in 2 patients (5.27%). Neurological symptoms improved in 34 patients, remained stable in 2 patients and were aggravated in 2 cases during follow-up. In addition, there was a notable difference between the location of tumors and syringomyelia (P < 0.05). Syringomyelia occurred more frequently in the cervical segment than in any other spinal segment. Moreover, there was an association between symptom duration and clinical prognosis (P < 0.05). Ordinal regression analysis showed that the prognosis of middle duration groups (6-12 months) was better than early groups (0-6 months, p < 0.05, OR 20.21, 95%CI 2.34-336.97) and late groups (>12 months, p < 0.05, OR 11.54, 95%CI 1.30-102.21). Syringomyelia collapse or reduction occurred between two weeks and 15 months after surgery. An improvement of spinal function grade after surgery was more significant in syringomyelia reduction groups (p < 0.05). CONCLUSIONS The prevalence of syringomyelia due to SH is considerably high, and the initial clinical presentation of syringomyelia resulting from SH should be emphasized. Satisfactory outcomes were achieved by effective surgery in affected patients.