BACKGROUND/AIMS: Ligneous lesions are rare diseases characterized by fibrin deposition due to plasminogen deficiency and they may affect the mucosal areas as conjunctivae and gingiva. The course of this disease is progressive and typically results in early tooth loss. In this study, we aimed to evaluate the demographic, clinical, and histopathological features and treatment approaches for ligneous periodontitis lesions in the literature with additional cases of our own.MATERIALS AND METHODS: Two patients, siblings, were referred to our clinic with nodular and fragile gingival overgrowth on both jaws. Brown -yellow gingival growths which were prone to bleeding and covered the teeth were observed. While one of the siblings received pharmaceutical treatment, the other received surgical treatment. A literature search was performed to congregate articles using the PubMed database based on the following terms: [(ligneous periodontitis) OR (ligneous)] OR [(ligneous periodontal disease)] AND [English (Language)]. Lesions which were not located on the gingiva were the exclusion criterion.RESULTS: The literature review revealed 72 cases of ligneous gingival lesions during the years 1973-2020. All lesions found were classified in tables according to their characteristics, treatment approaches, recurrence rates, and follow-up periods. Ligneous lesions are rare diseases characterized by fibrin deposition due to plasminogen deficiency and they may affect the mucosal areas.CONCLUSION: It has been reported that there is still no consensus on treatment for these lesions. More studies are needed in the future in order to provide effective periodontal treatment for cases of ligneous periodontitis.