© 2019 Background: Spontaneous isolated carotid artery (CA) or vertebral artery (VA) dissection in the absence of coarctation has rarely been reported in the literature. We report the case of a 20-year-old woman with Turner syndrome (TS) who developed an acute left middle cerebral artery territory ischemic stroke from a spontaneous left internal carotid artery (ICA) dissection. We also conducted a systematic review of the literature to identify prior studies establishing an association or other case reports of isolated CA or VA dissection in TS. We queried 5 databases: MEDLINE (PubMed), Scopus, Embase, Cochrane Central, and CINAHL EBSCO. We used a standardized search clause across databases. Inclusion and exclusion criteria were applied to articles retrieved. Studies were excluded based on title alone, abstract, or after vetting the data presented in the paper. Case Description: Three case reports of patients with TS presenting with spontaneous intracranial and/or extracranial dissection of the ICA or VA were identified and included in this review. Conclusions: We present a case of bilateral spontaneous dissection of the ICA in a patient with TS. Only 3 reported cases of spontaneous extra- or intracranial dissection of the CA or VA were identified via a systematic review of the literature. Arterial dissection of the CA or VA, especially in absence of aortic coarctation, in individuals affected with TS suggest the possibility of systemic vasculopathy. More research is needed to establish a better understanding of the phenotypic effects of TS in macro- and microvascular structures.