Objective: There are only limited epidemiological studies evaluating the association between vertebrobasilar dolichoectasia (VBD) and outcomes. This study was designed to elucidate the outcome and prognosis of adults diagnosed with VBD by magnetic resonance angiography (MRA) and to ascertain if these outcomes were independent of known vascular risk factors. Methods: A cohort study was designed to compare VBD cases identified retrospectively from a computerised database of MRA reports with age and sex matched controls evaluated after a 4-7 year period, and 1440 MRA reports were reviewed. The inclusion criteria were age ≥18 years and a radiological diagnosis of VBD. Patients were excluded if there was haemodynamically significant stenosis or occlusion of the posterior circulation. Data were obtained by medical record review and telephone questionnaires. The primary outcome measure was transient or fixed posterior circulation dysfunction (PCD), with a secondary outcome measure of all cause mortality. Results: Sixty four VBD cases were obtained, and 19 cases (30%) were excluded due to refusal and/or insufficient follow up data. From the same computerised database, 45 controls were selected by consecutive sampling. The mean age at follow up was 73.4 years for VBD cases and 73.1 years for controls, with a median follow up period of 64 months. VBD was associated with fixed/transient PCD (p = 0.0001; estimated adjusted odds ratio (OR) of 20.6 and confidence interval (CI) of 4.4 to 95.3), and with all cause mortality (OR = 3.6 CI 1.3 to 10.3); (p = 0.018). VBD cases had 36% mortality, with 50% occurring within 34 months of the initial diagnosis. The VBD cumulative survival curve was statistically different from the controls (p = 0.012 by Mantel-Cox log rank test). Conclusions: This study suggests that VBD may be an independent risk factor for stroke. VBD cases had an increased likelihood for PCD, all cause mortality, and reduced cumulative survival independent of other vascular risk factors in this cohort. Larger population based prospective studies are required to verify these results.