In 1914, the German physicians Volhard and Fahrintroduced the name ‘maligne Form der Hypertonie’for a syndrome consisting of a severe elevation in bloodpressure (BP) accompanied by signs of acute end-organdamage [1]. They noted that neurological signs andsymptoms were often part of the clinical picture andemphasized the serious prognostic importance ofcertain fundoscopic changes. By analogy the term‘malignant hypertension’ was applied by Keith andWagener in 1921 when they observed papilloedemain patients with severe hypertension [2]. In 1928,Oppenheimer and Fishberg described a 19-year-oldcollege student who suffered from severe hypertensioncoinciding with repeated bouts of headache, neurolog-ical deficits and convulsions and first used the term‘hypertensive encephalopathy’ (HE) [3]. Although thecharacteristic retinal signs of malignant hypertensionwere not present at presentation but developed later inthe course of the illness, fundoscopy became a veryuseful tool in the diagnosis of HE. Nowadays bilateralretinal haemorrhages and/or exudates are also includedin the definition of malignant hypertension becausefurther research has indicated that survival rates ofpatients with haemorrhages and/or exudates with orwithout additional papilloedema were equal [4,5].The presence of severe acute end-organ damage decideswhether a patient needs a carefully controlledimmediate reduction of BP [6]. In this report wedescribe three patients who suffered from HE, yet inwhom the classical retinal signs of malignant hyper-tensionwerelacking.ThediagnosisHEcould,however,be confirmed quickly by the use of recently developedtechniques in cerebral imaging.