Posterior reversible encephalopathy syndrome (PRES) is a neuroradiological disorder primarily affecting the posterior circulation of the brain. It manifests with a spectrum of symptoms, typically accompanied by hypertension, headache, seizures, and blurred vision. The etiology of PRES remains uncertain, but it is believed to be associated with vasogenic edema. The underlying conditions can range from sepsis to drug-induced states, autoimmune diseases, systemic lupus erythematosus (SLE), which is the most prevalent connective tissue disease associated with PRES. SLE is a multisystemic disorder that impacts various organs in the body, with musculoskeletal and skin systems being the most affected. The morbidity and mortality associated with SLE stem from its potential involvement of life-threatening organs, including the central nervous system, kidneys, and respiratory system. This article will focus on the neurological impact of PRES, including its manifestations. The association between PRES and SLE will be discussed in detail, accompanied by a literature review that explores the variability of presentation, reversibility, and prognosis.