Objective　To explore the clinical manifestations,pathological characteristics and therapeutic responses of lipid storage myopathy (LSM).Methods　The clinical information of 22 LSM patients were collected and analyzed retrospectively.Results　Proximal limb muscle weakness and motor intolerance were observed in all patients.The concentrations of creatine kinase in blood were found increased to various degrees in 20 cases.EMG examinations showed myogenic damage in 18 cases.Muscle pathology examination of 22 cases showed increased lipid droplets in the muscle fibers.Follow-up data was available for 20 of all the patients after treatment.Nineteen cases were treated with low-doses prednisone,18 cases with riboflavin and 6 cases with L-Carnitine.All patients showed distinct degree of improvement of clinical symptoms within a month.After treatment for 3 months,8/22 patients were recovered except for 2 patients who were relapsed.And repeated treatment was effective.Conclusions　LSM is a curable disease.It is recommended to avoid fever,fatigue and other triggering factors.In the absence of genetic testing,riboflavin,L-carnitine,and prednisone should be used empirically.In the presence of genetic testing,targeted therapy should be performed based on gene mutations.