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Background: Misdiagnosis in Cervical Artery Dissection (CAD) is thought to be uncommon but presenting symptoms and risk factors for misdiagnosis are not well defined. Methods: A nested cohort of patients with acute nontraumatic CAD were identified from a single center (2015-2021). Patients with CAD originally misdiagnosed (visiting a medical provider within 30 days for symptoms related to CAD without diagnosis of CAD) were compared against those diagnosed at presentation using descriptive statistics and unadjusted logistic regression. Results: Of the 52 included patients, 25 (48%) had a stroke at diagnosis, and 14 (27%) were misdiagnosed as non-CAD. Patients misdiagnosed had less severe pre-existing disability (median modified Rankin Scale and interquartile range [IQR], 0 [0-1] vs. 1 [0-1]) and were non-significantly younger (median 45 [35-68] vs. 53 [40-65], p=0.35). There was no difference in the distribution of vascular risk factors between groups. The most common presenting symptoms in misdiagnosed patients, such as focal deficits (43% vs. 42%, p=0.96) and headache (36% vs. 21%, p=0.78) were not associated with misdiagnosis. Less frequent symptoms like Horner’s syndrome (21% vs. 5%, p=0.11), neck pain (21% vs. 8%, p=0.33), and dizziness (14% vs. 5%, p=0.29) were non-significantly more common among patients with misdiagnoses. Conclusions: One-quarter of patients with nontraumatic CAD were misdiagnosed at first presentation. Misdiagnoses may be more common in younger persons with less pre-existing disability, and those with nonspecific symptoms such as dizziness and neck pain. Larger studies are needed to provide more precision in estimates of association.