Genetic testing for CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) and MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) was negative, but a heterozygous c.937G>T (p.Asp313Tyr) variant in the GLA gene was detected, indicating Fabry disease. Here, GLA is linked to mitochondrial encephalomyopathy.