We report a Southern Min Chinese family with two siblings carrying the c.250G > A (p.Ala84Thr) mutation in the ETFDH gene who presented with typical features of adolescent-onset riboflavin responsive multiple acyl-CoA dehydrogenase deficiency (MADD) associated lipid-storage myopathy. The gene discussed is ETFDH; the disease is multiple acyl-CoA dehydrogenase deficiency.