Introduction: The injuries on skeletal muscle that go beyond their capacity to adaptation result in characteristic pathological changes of the myopathies. In Colombia for their diagnosis is usually used the hematoxylin-eosin stain, however, the information about many changes in muscle tissue is limited. That is why it is necessary to carry out certain enzymatic histochemical staining according to each myopathy. Objective: To describe the different criteria to diagnose myopathies by histochemical techniques. Material and methods: A systematic review was conducted which focused on search of all articles written in English and Spanish indexed in ISI Web of Science database, Ovid Medline, PubMed, ScienceDirect, and EBSCO. The MeSH descriptors used for the search were “histochemistry”, “skeletal muscle”, “structural and congenital myopathies”, “fast twitch fiber type”, “slow twitch fiber type”, and “NADH tetrazolium reductase”. The terms “clinical application” and “diagnosis” were associated to the search. All original articles, case reports, and reviews published during 1982-2014, which implemented histochemical techniques to the diagnosis of myopathy or to biomedical research were included. Results: From systematic search, 64 publications (including two books) were representative for the drafting of this manuscript. The article was supplemented with images from muscle biopsies processed by the Laboratory of Histology, Universidad del Valle, Colombia. Conclusions: Standardization of protocols of histochemical techniques, together with pathologic criteria, can guide the diagnosis of myopathies in a timely manner and at low-cost compared with other available techniques.