Ligneous conjunctivitis is a rare chronic disease that is characterized with woody pseudomembrane formation in conjunctiva. It is usually due to autosomal recessive inheritance of type 1 plasminogen deficiency. Plasminogen deficiency results in impaired fibrinolysis and wound healing. Minor traumas trigger the formation of intense fibrin and inflammatory cell membrane deposits. These membranes may appear on conjunctiva or other mucosal surfaces in the body. Despite the use of topical cyclosporin, fresh frozen plasma and thrombin inhibitor, recurrences can be observed. In this article, we aimed to present a diagnostic and therapeutic approach for a 6-year-old ligneous conjunctivitis. His serum plasminogen level was low (3.4 mg/dl). Histopathological examination of expelled membranes revealed dense inflammatory cellular infiltrate with neutrophil dominance with eosinophilic amorphous material accumulation. Topical 0.1% dexamethasone and 2% high concentration of cyclosporine treatment were well tolerated and no recurrence was observed at 4 years follow-up. The etiologic factors should be investigated in mindful and suspicious cases where the conjunctivitis table may be indicative of a systemic disease.