Pain in the masticatory muscles or temporomandibular joints may in some cases be a symptom of other afflictions occurring in this region. The aim of the study was to present the differential diagnosis of temporomandibular disorders (TMD) and other diseases in the craniofacial area, based on review of the literature. Using the key words: "differential diagnosis of TMD", "pain of non-dental origin" and "chronic orofacial pain", PUBMED and Scopus databases were systematically searched for articles in English from 2005 to 2020. Additionally, the PUBMED database was supplementarily reviewed using the keywords "Lyme disease orofacial symptoms" for the English-language articles published in the years 1996-2020. Out of 445 publications from PUBMED and Scopus databases as well as other sources, 57 articles describing the pathogenesis and characteristic symptoms of diseases that may cause pain similar to that occurring in TMD as well as diagnostic methods used in differential diagnosis of TMD were selected for analysis. Dental and jawbones-related conditions, ear and maxillary sinus diseases, as well as ailments of neuropathic and vascular origin, were taken into account. Neoplastic processes taking place in this region and less often occurring diseases caused by viruses, bacteria and parasites were also described. Conclusions. Correct diagnosis of temporomandibular disorders is based on medical history and thorough physical examination, as well as results of additional tests. Pain localized in the head and neck structures may have diverse, sometimes complex aetiology, and may require multidisciplinary treatment. Observation of the patient's behaviour and – in selected cases – the results of additional laboratory tests, also play a significant role.