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AIM: To investigate the magnetic resonance imaging (MRI) findings of lipidstorage myopathy(LSM), evaluate the clinical value of MRI for LSM.METHODS: Eight cases with LSM biopsy-proved underwent MRI of pelvisand thigh, spin echo sequence, fast spin echo sequence and short tau in-version recovery(STIR) were utilized. RESULTS: The signal intensity ofdiseased muscle of 8 cases with LSM was hyperintense on T2-weighted im-ages (T2WI) and T1-weighted images (T1WI), and hyperintense signal in-tensity on T2WI and iso-intense on T1WI was seen on 2 patients as well .CONCLUSION: MRI can provide objective data for clinical diagnosis,therapeutic evaluation and follow-up. It can also help to decide the accuratelocalizations for biopsies.