Objective To investigate the cause of elevated serum muscle enzyme by analyzing the pathological diagnosis in patients with simple elevation of serum muscle enzyme.Methods The demographic data, serum muscle enzyme levels, electromyography, and pathological characteristics of 24 patients with simple elevation of serum muscle enzyme were subjected to inductive analysis.Results There were 16 males and 8 females, who were aged from 3 to 54 years, and the mean age of onset was (32.56±15.93) years. Creatine kinase levels were 577.40-17720.00 U/L (mean value: 8081.83±6065.50 U/L) and lactate dehydrogenase levels were 150.20-1643.80 U/L (mean value: 415.30±324.90 U/L). Electromyography of 20 patients showed that 15 had myogenic damage, 3 were normal, one was suspected of myogenic damage, and one had shortened motor unit time. Muscle pathology revealed that 2 cases (8.33%) were normal, 5 (20.83%) had nonspecific scattered muscle atrophy, and 17 (70.83%) had myopathy, including 13 cases (54.17%) of muscular dystrophy, 3 cases (12.50%) of inflammatory myopathy, and one case (4.17%) of Danon disease.Conclusions There are many causes of asymptomatic elevation of muscle enzyme, and muscle enzyme histochemistry and immunohistochemistry are conducive to the diagnosis in most patients.