<h4>Introduction</h4>Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is a rare mitochondrial disorder primarily presenting with neurological manifestations, but it may also lead to gastrointestinal complications such as intestinal pseudo-obstruction, which can significantly complicate clinical management.<h4>Case presentation</h4>We report a 24-year-old woman with genetically confirmed MELAS (mtDNA A3243G mutation and TRPM3 c.2878G > T heterozygous variant) who was admitted with acute confusion and generalized convulsive seizures. Here, TRPM3 is linked to mitochondrial encephalomyopathy.