Laryngeal amyloidosis is a rare and idiopathic disease that represents about 1% of all benign laryngeal lesions. It is characterized by the extracellular deposition of an abnormal amount of non-soluble fibrillar proteins in larynx. This disease presents unspecific symptoms and laringoscopic findings that make difficult to diagnose it. Management of these lesions consists of endoscopic resection of the mass through microdirect laryngoscopy. Recurrence is possible and it can occur up to 10 years after treatment; therefore a close and long-term follow-up is requested. A 60-year-old man presented with hoarseness and dysphonia. Laryngoscopy revealed a smooth, translucent and yellowish formation involving the middle-third of the left true vocal cord. The patient was first managed conservatively with speech therapy and after 6 months the lesion was removed by endoscopic CO2 laser surgery without evidence of recurrence in 2 years of follow-up. Laryngeal amyloidosis is a rare disorder that can be mistaken for a benign vocal cord polyp, nodule, retention cyst or laringocele. CO2 laser technique represents the highly effective approach to treat these lesions. Clinicians should know this rare amyloidosis site in order to avoid a delayed diagnosis and to treat it.