Congenital plasminogen deficiency is a rare autosomal recessive disorder that affects approximately 1.6 per one million people. Impaired fibrin degradation and diminished wound healing lead to thick woody deposits or mucosal pseudomembrane formation that may involve almost any organ system. Lesions most commonly form on the conjunctiva resulting in the disease’s hallmark manifestation, Ligneous Conjunctivitis however laryngeal involvement is seldom reported in the literature. We present the case of a 55 year old male with congenital plasminogen deficiency leading to glottic lesions of adult onset. The patient initially presented with subacute dysphonia in the setting of a high vocal load occupation. Stroboscopy showed bilateral membranous vocal fold lesions with further airway assessment showing a posterior mid-tracheal lesion. Histopathology was non-diagnostic and the lesions were refractory to phonomicrosurgical resection with recurrent bulky vocal fold granulation visible within weeks of surgery. Further serial intra-lesional steroid injections were of limited therapeutic benefit, and so too, extensive voice therapy and judicious occupational vocal load reduction. A diagnostic dilemma, the patient recalled suffering from a rare ocular condition, Ligneous Conjunctivitis as a toddler, which had left him with long term visual impairment. Subsequent plasminogen levels confirmed the diagnosis and systemic therapy was initiated with intravenous fresh frozen plasma (FFP) infusions. This treatment failed to regress the established lesions, and so, repeat phonosurgical resection under plasminogen cover was performed. Unfortunately, recurrent lesions appeared, and thus a plan for a novel administration method for local FFP was devised. This is a rare case of congenital plasminogen deficiency with laryngeal involvement. A challenging case from a diagnostic and management perspective, the rarity of this cause for glottic lesions necessitated the use of treatment options outside the routine treatment paradigm.