Aim: Report a case of a very rare clinical presentation of syphilis, manifesting exclusively in the larynx. Background: Syphilis is a sexually transmitted disease, usually with multisystemic involvement, that has seen an increase in incidence over the past two decades. The disease can present in four different stages and can mimic many other diseases. Laryngeal manifestations are rare and can result from different mechanisms, such as direct damage to the larynx, vagal neuropathy, and central nervous system dysfunction. Syphilis can affect the larynx along its course, but an isolated manifestation of syphilis in the larynx is extremely rare, with only few cases reported in the literature. Case description: We present a case of a 45-year-old man who initially presented with an isolated lesion in the aryepiglottic fold which was biopsied and diagnosed as a syphilitic lesion. Despite adequate medical treatment, he progressively developed a bilateral vocal fold immobility requiring tracheostomy, and subsequently a laser posterior cordectomy for definitive treatment. Conclusion and clinical significance: The global incidence of syphilis is increasing, and although it can have laryngeal manifestations, an isolated presentation of syphilis in the larynx is extremely rare. Therefore, a high index of suspicion is needed by otorhinolaryngologists for a timely diagnosis and treatment in order to prevent the sequelae of this disease.