Ligneous mucosal disease is a rare, destructive condition of plasminogen deficiency characterized by generalized membranous gingival enlargement leading to rapid tooth loss. We report a case of long-standing, generalized ligneous periodontitis with an eleven years of follow-up. The patient did not have any extra oral lesions although she had been suffering from plasminogen deficiency. Treatment approaches had no benefit. An abnormal healing process is the main pathogenetic mechanism; still, intensive periodontal health care is essential for the patients who suffer from ligneous mucosal disease. This report describes a ligneous periodontitis case with a history of ligneous conjunctivitis and its clinical and histopathologic findings, therapeutic approaches and eleven years of follow-up.