The authors describe a patient with personal and family history of muscle pains highly suggestive of mitochondrial disease. She presented with familial combined hyperlipidaemia with family history of premature coronary artery disease. After 4-week therapy of 5 mg of rosuvastatin once a week combined with 10 mg of ezetimibe daily, the low-density lipoprotein cholesterol level was markedly decreased from 3.88 to 2.82 mmol/l (27.3% reduction). She has experienced no adverse effects after 6 month treatment. The results suggest that the combination therapy of low-dose once-weekly rosuvastatin and daily ezetimibe could be used as an effective regimen for patients with mitochondrial disease.