Transport and Golgi Organization Protein 2 Homolog (TANGO2)-related disease is a pathology with a poor prognosis due to the recurrence of severe life-threatening rhabdomyolysis (RM) bouts 1,2, characterized by the acute break-down of skeletal myofibers.3,4 Other symptoms of TANGO2 disease are developmental regression, hypoglycemia, hypothyroidism and cardiac arrhythmia, including prolonged QTc interval or ventricular fibrillation.1,2,,5–12 RM can be triggered by fasting and infections, but also by exposure to cold or heat. Here, TANGO2 is linked to Hypoglycemia.