Autoimmune vasculitides can have diverse neurological manifestations, including posterior reversible encephalopathy syndrome (PRES). Takayasu's arteritis (TA) is an uncommon vasculitis rarely associated with PRES. Common clinical features of TA include hypertension, audible arterial bruits, absence of peripheral pulses, claudication of the extremities, reduced blood pressure in one or both arms, and angiographic abnormalities. PRES has been mostly associated with severe hypertension, endothelial injury, and conditions such as renal disease, immunosuppressive medication use, and rheumatologic diseases. Headaches, seizures, and altered mental status are the main clinical features as well as characteristic findings in magnetic resonance imaging. TA frequently presents with hypertension and is associated with endothelial injury, making this entity an ideal setting for the development of PRES. We report the case of a 17-year-old female who presented to the emergency department with severe hypertension, headache, and seizures. Magnetic resonance imaging findings were suggestive of PRES. She had absent pulses in the right upper extremity, abdominal bruits, and angiographic findings included subclavian and renal artery stenoses. The diagnosis of TA was made, and she responded well to treatment. We found ten additional cases of TA and PRES in the literature. All patients were females under the age of 40, had renovascular hypertension, and presented with headaches and seizures. Current literature relevant to this rare association is presented and discussed.