To provide practical guidelines for handling muscle biopsies to be submitted for outside processing and consultation.Review each step of handling a muscle biopsy and provide alternative means of tissue processing when feasible.Practical guidelines are presented for specimen selection, performance of needle biopsy, preparation of tissue for electron microscopy, preparation of tissue for shipping, clinical information to be provided, and submission of tissue for molecular and biochemical studies.Muscle biopsy samples to be sent to referral centers can be properly handled to avoid the problems noted by the Neuropathology Needs Assessment of the College of American Pathologists.