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Introduction: Temporomandibular joint (TMJ) septic arthritis is a rare frequently misdiagnosed condition with non-specific symptoms. We present our experience of thirteen cases of TMJ septic arthritis and perform a systematic review of the literature to collate the multiple characteristics of this condition., Material and Method: A total of 133 cases of TMJ septic arthritis in humans across 62 studies were analyzed by searching PubMed, Cochrane Library, DOAJ and ClinicalTrials.gov using the following search terms: "TMJ septic arthritis," "Temporomandibular septic arthritis," "TMJ infectious arthritis," and "Temporomandibular infectious arthritis.", Results: We identified three routes of TMJ septic arthritis dissemination: the hematogenous route, direct inoculation, and local contiguity. Joint and rheumatic pathologies and immunomodulatory diseases are risk factors. The most frequently causative bacterial genus is Staphylococcus, followed by Streptococcus. Causative bacteria can be identified by bacteriological analysis. Magnetic resonance imaging, computed tomography (CT), and scintigraphy can be used for diagnosis, but CT is the gold standard in an emergency setting. Blood tests often reveal a high C-reactive protein concentration and high leukocyte counts. Signs and symptoms include preauricular swelling and trismus, and, less commonly, fever, ipsilateral hemifacial pain, joint disorder, and malocclusion with mandibular deviation. Timely treatment is key to avoid short and long-term complications, because proteolytic enzymes from granulocytes can cause irreversible damage within 7 days. Antibiotic therapy, arthroplasty, and physiotherapy are commonly used treatment modalities., Conclusion: TMJ septic arthritis can be misdiagnosed due to its non-specific clinical manifestations. Complications can occur; thus, timely and effective treatment is key., Competing Interests: Declarations. The authors did not receive support from any organization for the submitted work and did not have no relevant financial or non-financial interests to disclose., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)