Organic acidurias (OAs) belong to the group of inborn errors of metabolism (IEM) caused by enzymatic or transport proteins defects in the metabolic pathways of the amino acids, lipids or carbohydrates, having as consequence an energetic failure due to the insufficient “fuel” for the cycle of Krebs and the accumulation of intermediate metabolites with a deleterious effect on different organs, including the nervous system. Diagnosis is based on detection of intermediate metabolites in body fluids, on genetic analysis and measurement of the enzymatic activity in cultured fibroblasts. Strokes related to OAs belong to metabolic stroke category for glutaric, isovaleric, methylmalonic (MMA) and propionic acidurias (PA) and is related to clot formation in the dilated cardiac chambers and to infections favored by neutropenia, in Barth syndrome. Basal ganglia are frequently affected, leading to various types of movement disorders. Both ischemic and hemorrhagic strokes may occur. Early diagnosis, prompt initiation of specific diets, adequate treatment of acute decompensations and chronic monitoring of the patients can reduce the mortality and disability