BACKGROUND AND OBJECTIVE: Treatment of type 1 diabetes mellitus (DM) aims to prevent complications by strictly optimizing blood glucose levels. Although physical exercise is an important part of metabolic control, endurance sports are considered hazardous for patients with type 1 diabetes because of the extreme physiological stress they represent. To further elucidate the metabolic challenge this form of exercise presented we investigated the performance of triathlon competitors with type 1 diabetes. PATIENTS AND METHODS: Ten patients (32-61 years) with type 1 diabetes (disease duration 2-35 years) were followed for three years, during which each year they participated in one triathlon long-distance competitions (2.4 miles swimming, 26.2 miles running and 112 miles cycling; Ironman Germany 2005-2007). Glucose, cortisol, aldosterone, renin, thyroid hormones, testosterone, growth hormone and catecholamines were measured in blood and saliva. Five non-diabetic competitors served as controls. RESULTS: The performance equalled those of age-matched healthy athletes. Several participants experienced hyperglycemia early in the bike leg, whereas all of them developed low blood glucose levels during the marathon leg. Basal insulin supply was reduced up to 50 % on race day. Hormone levels in athletes with type 1 DM and healthy controls were similar. CONCLUSIONS: Patients with type 1 DM can successfully sustain extreme endurance challenges. Physiological alterations of the metabolic state complicated by type 1 DM can readily be compensated by adapting intensified insulin therapy and nutritional modifications. Thus 1 DM should not be regarded a contraindication to participating in high endurance sports.