Posterior reversible encephalopathy syndrome (PRES) is a rare condition manifested by inflammation in certain areas of the brain. Rhabdomyolysis with acute kidney injury (AKI) complicated by PRES is rarely reported. A 26-year-old female presented with neurological symptoms, high blood pressure, and AKI. Her symptoms improved with blood pressure control, anticonvulsant drug medications, and renal replacement therapy. This case demonstrates that PRES should be considered in the differential diagnosis of patients who have rhabdomyolysis with AKI accompanied by neurological symptoms, including headaches and convulsions.