The appearance of motor clumsiness, with running and jumping difficulties in a 6-year-old boy, prompted the genetic study of Friedreich’s ataxia, confirming his diagnosis. After diagnosis, it was evaluated by Pediatric Cardiology, detecting the presence of non-obstructive hypertrophic cardiomyopathy, and by Pediatric Endocrinology, due to overweight. At 9 years of age, he was diagnosed with diabetes mellitus(Diabetes mellitus and Friedreich´s ataxia), and a regimen of insulin treatment was initiated. During follow-up, he presented significant neurological deterioration, reaching the use of a wheelchair, which hinders adequate metabolic control.