Abstract
Primary mitochondrial diseases are a heterogeneous and complex group. Usually, they are a congenital disorder of energy metabolism and ATP production.
The first named mitochondrial disease is MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes) caused by mitochondrial DNA mutation. MELAS syndrome is very often associated with the presence of diabetes mellitus.
The case report describes a female patient with mitochondrial diabetes, and also tries to determine when it is appropriate to consider the disease in common practice of a diabetologist and how to choose the right treatment.