Objectives: To evaluate the etiopathogenesis, clinical features, therapeutic options, and surgical approaches for removal of ectopic third molars in the mandibular condyle. Study design: MEDLINE search of articles published on ectopic third molars in the mandibular condyle from 1980 to 2011. 14 well-documented clinical cases from the literature were evaluated together with a new clinical case provided by the authors, representing a sample of 15 patients. Results: We found a mean age at diagnosis of 48.6 years and a higher prevalence in women. In 14 patients, associated radiolucent lesions were diagnosed on radiographic studies and confirmed histopathologically as odontogenic cysts. Clinical symptoms were pain and swelling in the jaw or preauricular region, trismus, difficulty chewing, cutaneous fistula and temporomandibular joint dysfunction. Treatment included conservative management in one case and in the other cases, surgical removal by intra- or extraoral approaches, the latter being the most common approach carried out. In most reported cases, serious complications were not outlined. Conclusions: The etiopathogenic theory involving odontogenic cysts in the displacement of third molars to the mandibular condyle seems to be the most relevant. They must be removed if they cause symptoms or are associated with cystic pathology. The surgical route must be planned according to the location and position of the ectopic third molar, and the possible morbidity associated with surgery.