Abstract Mitochondrial Neurogastrointestinal Encephalomyopathy (MNGIE) is a rare autosomal-recessive disorder which is due to mutations in TYMP. The case reported here is of an 18-year-old male with MNGIE syndrome who presented for two different operations on two different occasions under regional anesthesia. The patient presented with urinary incontinence and abdominal pain. A cystoscopy under spinal anesthesia was scheduled. At 3 months after discharge, gastric perforation was diagnosed and combined spinal-epidural anesthesia, surgical repair was planned. Surgical and perioperative periods were uneventful. Based on this experience, we believe that regional anesthesia can be considered safe for use in patients with MNGIE disease.