Aim: A number of systemic disorders increase patient susceptibility to periodontal disease by altering the immune, endocrine, and connective tissue status. This case report describes the periodontal manifestation, treatment and long term maintenance of a patient diagnosed with Ehler Danlos Syndrome (EDS) type VIII. EDS VIII is a rare autosomal dominant connective tissue dysplasia characterised by fragile skin, abnormal scarring, joint hypermobility, and early onset of periodontal disease. In addition, loss of the permanent teeth may also be detected. Case Report: A 28-year-old Caucasian female was first introduced in 1999, intra oral examination revealed localized areas of moderate to high attachment and alveolar bone loss (mandibular incisors) associated with mobility grade II; probing depths ranged between 2 and 3.5 mm. The gingival tissues exhibited generalised gingivitis with spontaneous bleeding and massive gingival recession. The treatment consisted of oral health measures followed by regular supra gingival scaling every three months which continued over the following years. Result: Over the subsequent years and the extraction of the hopeless lower incisors, no further tooth loss was recorded in the remaining teeth. Conclusion: Early diagnosis, efficient periodontal therapy, and long-term maintenance applied to patients suffering from EDS VIII are the key elements of preventing further tissue destruction and tooth loss. Keywords: Ehlers Danlos Syndrome, Periodontitis