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While cerebral vasculopathy is a well-known early complication of sickle cell disease that has warranted transcranial doppler surveillance, little has been documented regarding the role of cervical circulation in the development of cerebrovascular events and transient ischemic attacks amongst sickle cell patients. We present the unique case of a symptomatic 18-year-old male with sickle cell disease found to have critical cervical carotid stenosis in the absence of significant intracranial vasculopathy. Increased awareness of the potential role of the extracranial circulation in the development of sickle cell related cerebrovascular events is warranted.
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