ABSTRACTThere are some indications that seizure activity promotes the development of stroke‐like episodes, or vice versa, in patients with mitochondrial encephalopathy, lactic acidosis and stroke‐like episodes (MELAS) syndrome or other syndromic mitochondrial disorders. A 41‐year‐old Caucasian female with MELAS syndrome, presenting with short stature, microcytic anaemia, increased blood‐sedimentation rate, myopathy, hyper‐gammaglobulinaemia, an iron‐metabolism defect, migraine‐like headaches, and stroke‐like episodes, developed complex partial and generalised seizures at age 32 years. Valproic acid was ineffective but after switching to lamotrigine and lorazepam, she became seizure‐free for five years and stroke‐like episodes did not recur. Cerebral MRI initially showed enhanced gyral thickening and a non‐enhanced T2‐hyperintensity over the left parieto‐temporo‐occipital white matter and cortex and enhanced caudate heads. After two years without seizures, the non‐enhanced hyperintense parieto‐temporo‐occipital lesion had disappeared, being attributed to consequent seizure control. The caudate heads, however, remained hyperintense throughout the observational period. This case indicates that adequate seizure control in a patient with MELAS syndrome may prevent the recurrence of stroke‐like episodes and may result in the disappearance of strokelike lesions on MRI.