Background & Aim: Intramedullary spinal cavernoma (ISC) is a rare entity and accounts for approximately 5%– 12% of all spinal vascular pathologies. The purpose of the present study was to examine the influence of clinical presentation, localization, and different surgical approaches on long-term outcome in patients treated for I SC.Methods & Materials/Patients: The authors performed a retrospective single-center study of 18 cases treated microsurgically over the past 8 years. Analyzed factors included preoperative clinical history, microsurgical strategies, neurological outcome (American Spinal Injury Association [ASIA] grade, Epstein and Cooper grade), and the occurrence of postoperative spinal ataxia. Univariate analysis was performed to identify factors influencing long-term outcome.Results: Preoperatively, 18.8% of all patients experienced a slowly, progressive decline in neurological function and 33.3% suffered from repetitive episodes of acute neurological deterioration over a time period from months to years. Moreover, 16.7% noted the sudden onset of a severe neurological deficit, whereas 25% experienced the sudden onset of symptoms with a subsequence of gradually progressive decline in neurological function. On long-term follow-up after treatment (mean ± SD, 79.3 ± 35.2 months), 70.8% of patients showed no change in neurological function, 6.3% suffered from a decline, and 22.9% improved neurologically. Thoracolumbar localization (p=0.043), low preoperative Epstein and Cooper grade for the lower extremities (p<0.001), and a low preoperative ASIA grade (p<0.001) were identified as factors associated with an unfavorable outcome (ASIA Grade A–C). The rate of spinal ataxia related to surgical approach was 16.7%.Conclusion: Postoperative neurological function in ISC patients was determined by the preoperative neurological status. In the long-term follow-up after microsurgical treatment, 93.7% of patients presented with a stable or improved condition (ASIA grade); thus, definite microsurgical treatment should be considered as long as patients presented with only mild symptoms after the diagnosis of sympto matic ISC.