Purpose: To evaluate the etiopathogenesis, clinical features and surgical approaches for removal of ectopic third molars in the mandible. Methods: We report a case of an impacted mandibular third molar dislocated on mandibular sigmoid notch. 20 cases of ectopic mandibular third molars reported in the English-language literature, identified from Pubmed and Medline databases are also reviewed. Results: Among the 20 article reported in the presented study, ectopic third molars were generally located in the condylar region. The common symptoms of the clinical examination were pain, trismus, swelling, temporomandibular joint syndroms or no symptoms. Conclusions: Ectopic third molar may be asymptomatic initially with clinical manifestations, later on as adjacent structures are affected. The surgical approach must be carefully planned for the aim of choosing the more conservative technique that produces the minimum trauma to patients.