Malignant hypertension (MHT) is the most severe form of hypertension which is clinically defined as the presence of high blood pressure in association with bilateral retinal haemorrhages and/or exudates, with or without papilloedema. The aim of this review article is to discuss whether MHT is a problem which is truly becoming a rarity, or is it simply a problem with underdiagnosis. Despite the improvements in the general management of hypertension, we have no strong evidence of a declining incidence of MHT. In contrast, this disorder may appear to become even more common worldwide taking into account the growing hypertensive population in the developing countries. Although the diagnostic criteria of MHT appear to be simple and straightforward, the prompt diagnose of MHT may be difficult in substantial proportion of patients who often present with clinical symptoms only at a late stage of irreversible target organ changes. Furthermore, MHT and the accompanying ocular changes may gradually resolve making retrospective diagnosis problematic, whilst persistent target organ damage can drive the development of complications and have a negative prognosis in these patients. Clearly, MHT should not yet be forgotten nor ignored by clinicians.