RESUMO
Multiple acetyl CoA dehydrogenase deficiency is a rare autosomal recessive disorder of amino acid, fatty acid, and choline metabolism. It is a mitochondrial disorder with defective electron transfer flavoproteins or electron transfer flavoprotein dehydrogenases. They are vital for ß-oxidation of fatty acids, an essential fuel for skeletal and cardiac muscles. It is also an important source of energy during starvation for the brain. Acute deterioration of these patients can occur during stressful periods like starvation, surgery, infection, and exercise. The anaesthetic management is a challenge with special emphasis on minimizing starvation, ensuring hydration and glucose supplementation, and considering the various effects of anaesthetic agents on the mitochondrial function. The anaesthetic management of a patient with multiple acetyl CoA dehy- drogenase scheduled for modified radical mastectomy is described. General anaesthesia can be administered safely in these patients with special emphasis on hydration, glucose supplementation, avoidance of stressors, and monitoring of metabolic status.