Muscle biopsy is often required to provide a definitive diagnosis for neuromuscular disorders and can be performed using open surgical or percutaneous needle biopsy techniques. Fresh samples that are subsequently frozen in the laboratory are preferred by laboratories engaged in research, whereas formalin-fixed muscle is processed by diagnostic laboratories with specialized tinctorial and immunohistochemical techniques. Interpretation by an experienced histopathologist, combined with the signalment, history, and clinical input, is essential for establishing a diagnosis. This article outlines best practices to select, obtain, and ship a muscle sample to a laboratory and a summary of laboratory techniques.