We found a 43-year-old man proband carrying a rare homozygous ABCC6 p.E709K variant had infarcts at the bilateral corona radiata, bilateral basal ganglia, and pons because of vertebrobasilar artery dissection, which was phenotypic compatible with ABCC6 related pseudoxanthoma elasticum. The gene discussed is ABCC6; the disease is pseudoxanthoma elasticum (inherited or acquired).