An 8-year-old, right-handed male with recently diagnosed MELAS, presented with sudden onset vomiting, vision changes, fevers, and malaise, followed by intermittent episodes of acute cortical blindness. Fundus exam was normal with intact pupillary reflexes. During these episodes, patient was also noted to have Anton-Babinski Syndrome with inability to discern objects, color, or people around him with anosognosia and confabulation. Video Electroencephalography (V-EEG) monitoring showed multiple occipital seizures, emerging from the right occipital lobe and diffusing rapidly over the left occipital region. These electrographic seizures were associated with the episodes of cortical blindness , and the patient was intermittently regaining his vision with resolution of ictal pattern on V-EEG. MRI with and without contrast showed abnormal T2 FLAIR hyperintensity of the cortex and subcortical white matter of the occipital lobes, greater on the right with corresponding mild cortical restricted diffusion with no drop out signals on ADC. Constellation of findings related to postictal changes. His condition improved with adjustments of anti-epileptic drugs including administering lorazepam, continuing with levetiracetam and adding lacosamide as a second drug.