Current knowledge about venous hypertensive myelopathy (VHM) is incomplete. This study was performed with the aim of clarifying the clinical features and outcomes of craniocervical VHM.This retrospective, single-center cohort study included 65 patients with craniocervical junction arteriovenous fistulas resulting in VHM treated in Xuanwu Hospital from January 1, 2002, to December 30, 2020. All patients underwent microsurgery or endovascular treatment. The primary outcome was neurological function assessment using the Japanese Orthopaedic Association (JOA) scale, modified Aminoff-Logue Scale (mALS), and Venous Hypertensive Myelopathy Scale (VHMS). The secondary outcomes were recurrences and postoperative adverse events. Pearson linear regression and receiver operating characteristic curves were used to evaluate the relationships among the three scales. Kaplan-Meier and multivariate logistic regression analyses were performed to predict outcomes.The mean patient age was 57.4 ± 11.4 years, and 88% of patients were male. The 1-year follow-up rate was 83.1%, and the 5-year follow-up rate was 50.8%. The VHMS was correlated with the JOA (R2 = 0.6722) and mALS (R2 = 0.7399) and increased the assessment accuracy by approximately 20% when compared with the other two scales. Overall, 25.9% of patients experienced delayed neurological decline beyond the 1-year follow-up. Further logistic regression suggested that age > 65 years was an independent predictor (OR 7.831, 95% CI 1.090-56.266; p = 0.041). Embolic recanalization and new bilateral symmetry feeders were the major reasons for recurrence. Recurrence increased the risk of adverse events after the second surgery (OR 20.455, 95% CI 1.170-357.320; p = 0.039).CCJ AVFs resulting in VHM are a rare but deadly complication, and providers should be cautious of age-related delayed neurological decline and strive for a one-time anatomical cure.