Systemic elastorrhexis is a multisystem genetic disorder characterised by dystrophic mineralization of soft connective tissues in a number of organs, including the skin, the eyes and the arterial blood vessels. Although the eye and skin findings have for years attracted the attention of ophthalmologists and dermatologists, the systemic nature of the disorder has not received sufficient attention among internists and many patients with this disorder have undoubtedly been unrecognized. We reported a case of systemic elastorrhexis redressing the diagnosis of vascular leucoencephalopathy of an unknown aetiology for many years.