A 24-year-old woman with genetically confirmed MELAS (mt2838G mutation and TR2838G heterozygous variant >.3) was admitted for acute confusion and generalized convulsive seizures. CT showed multiple low-density lesions and MRI abnormal signals in the bilateral occipital lobes and basal ganglia. After stabilization of neurological symptoms with emergency treatment, repeated episodes of intestinal pseudoobstruction with vomiting and decreased bowel sounds developed. Conservative measures failed, so jejunal decompression was performed. This procedure resulted in a significant improvement in bowel function and transition to normal oral intake. The case illustrates delayed acute intestinal pseudo-obstruction as a complication of MELAS crisis, where early jejunal decompression can be effective.