Neuroborreliosis is a tick-borne condition that affects the central and/or peripheral nervous system. Cerebral infarction associated with neuroborreliosis-related vasculitis has been reported in only a handful of cases. Therefore, specific patterns of vascular pathology and prognostic outcome factors are still incompletely understood. To determine the pattern of vascular pathology and prognostic outcome factors in patients with neuroborreliosis-related vasculitis. We performed a longitudinal multicenter study between 1997 and 2022 in five academic study sites in Germany with a cumulative reference area of 1,620,000 inhabitants. All patients diagnosed with neuroborreliosis-associated cerebral vasculitis were included. The evaluation of clinical parameters, including NIH Stroke Scale (NIHSS), disability ranking (modified Rankin Scale, mRS), and neuroimaging, was performed at admission as well as after 3 and 12 months. Linear regression analysis was used to identify the independent predictors of recurrent strokes, involvement of posterior circulation, or multiple vessels. Patients with neuroborreliosis-related vasculitis (n = 51) were relatively young (mean age: 62 years) and displayed a predominance of vascular events within the posterior circulation (60.8%). A history of smoking was linked to recurrent strokes/TIA (64.7% vs. 23.5%; p = 0.006), strokes in multiple territories (100% vs. 35.9%; p < 0.0001), and posterior circulation events (64.5% vs. 30.0%, p = 0.017), whereas other cardiovascular risk factors showed no significant differences. Linear regression analysis corroborated smoking's association with recurrent strokes/ transient ischemic attacks (B: 0.412; p = 0.002), multiple territory strokes/TIA (B: 0.467; p = 0.033), and posterior circulation events (B: 0.317; p = 0.033). A thorough CSF examination for neuroborreliosis is crucial, especially in younger stroke patients, particularly those experiencing posterior circulation ischemic events. Smoking cessation should be prompted in patients with neuroborreliosis-associated cerebral vasculitis.