Trifid mandibular condyle (TMC) is a rare anatomical variation characterized by the duplication of the mandibular condyle. The aim of this study is to report a new case of a 26-year-old female patient with a left TMC and to review the current existing literature on TMC, the relevant cases, etiology, symptoms and different treatment modalities. The database engines PubMed, EMBASE, Scopus, Web of science, Scientific Electronic Library Online, Cochrane and CINAHL were searched for TMC cases from inception until April of 2022. Only 13 previous cases of TMC were found. Although it is a rare anatomical entity, TMC is increasingly being detected due to more advanced imaging techniques, especially computed tomography (CT), cone beam CT (CBCT) and magnetic resonance imaging (MRI) emerging in the field of dentistry. The etiology and pathogenesis of TMC and its relationship with TMD are still unclear. Further studies and follow-up may help to better understand this anatomic variant and possible interactions with local pathologies.