<b><i>Background and Purpose:</i></b> The diagnosis of cerebellar infarction (CBI) is often challenging due to non-specific or subtle presenting symptoms and signs. We aimed to determine whether a common syndromic cluster of symptoms, signs or vascular risk factors were associated with delayed presentation or misdiagnosis to an Emergency Department (ED). The degree of misdiagnosis between ED and neurology physicians and the influence of delayed presentation or misdiagnosis on outcome were also investigated. <b><i>Methods:</i></b> A prospective study of CBI patients at a large tertiary-referral hospital with a comprehensive stroke service. Data are reported with OR and 95% CIs. <b><i>Results:</i></b> Of 115 consecutive CBI patients (mean age ± SD 66 ± 14 years, 51% male), infarction was isolated to the cerebellum in 46%; the remainder had additional vascular territory involvement (‘mixed CBI'). Most patients (n = 79, 69%) had a mild stroke (National Institute of Health Stroke Scale score ≤4), and tended to present late to ED (>4.5 h; p = 0.05). Dysarthria (OR 3.9, 95% CI 1.6-9.6, p = 0.003) and prior history of atrial fibrillation (AF; OR 3.0, 95% CI 1.02-9.1, p = 0.047) predicted early presentation (<4.5 h; in 52%). Neurological signs (as determined by neurology physicians) were more commonly absent in patients with isolated CBI (OR 4.0, 95% CI 1.2-13.3, p = 0.03) who were also less likely to receive acute stroke therapy (p = 0.03). ED physicians detected fewer neurological signs than neurology physicians (mean 1 vs. 2 signs, p < 0.001), and 34% of CBI patients were misdiagnosed, with peripheral vestibulopathy being the most common alternative diagnosis. Nausea and vomiting (OR 2.3, 95% CI 1.01-5.5, p = 0.046), absence of neurological signs as determined by ED physicians (OR 3.5, 95% CI 1.5-8.0, p = 0.003) and isolated CBI (OR 2.2, 95% CI 1.01-4.8, p = 0.047) correlated with misdiagnosis. Vascular territory involvement did not correlate with time to presentation or misdiagnosis. At 3 months, 65% of patients were functionally independent (modified Rankin Scale (mRS) score 0-2). History of hypertension (p = 0.008), AF (p = 0.012), mixed CBI (p = 0.004) and in-hospital stroke-related complications (p < 0.001) were associated with patients having a poor outcome (mRS ≥3). At 3 months, mortality was 16%, and AF was the only predictor of death (OR 3.2, 95% CI 1.1-8.9, p = 0.03). Late presentation to ED and misdiagnosis did not significantly influence 3-month functional outcome. <b><i>Conclusions:</i></b> Late ED presentation and misdiagnosis are common for CBI. Timely diagnosis of CBI may increase opportunity for acute stroke therapies and reduce risk of stroke-related complications.