INTRODUCTION: Laryngeal tuberculosis (TB) is a granulomatous disease with a variable clinical presentation that can make diagnosis difficult. The larynx is an uncommon site of primary, extra pulmonary TB; on the other hand, TB is the leading cause of granulomatous lesions of the larynx. Most cases have successful treatment outcomes after the correct diagnosis is made and effective treatment started. OBJECTIVE: To emphasize the importance of TB as a cause of laryngeal lesions to promote early diagnosis and permit the prompt initiation of effective treatment conducive to a favorable outcome. CASE REPORT: A 46-year-old, single, Caucasian man working as a driver was referred to the otorhinolaryngology service of Hospital Geral da Santa Casa da Misericórdia do Rio de Janeiro with a 2-year history of progressive dysphonia and pain in the right side of the neck. The patient denied having had cough, fever, chills, or weight loss, or indulging in smoking or alcohol abuse. Videolaryngoscopy revealed ulcerative, granulomatous lesions in both vocal cords that were more extensive on the right side. Chest radiography showed no abnormalities and sputum culture was negative for M. tuberculosis. Histopathological and bacteriological examination of a biopsied specimen, confirmed the diagnosis of laryngeal TB. The patient was referred to the clinic nearest his home, where he began anti-tuberculosis therapy consisting of rifampicin, isoniazid, pyrazinamide, and ethambutol. He is presently on otorhinolaryngological follow up. CONCLUSION: Early diagnosis of laryngeal TB contributes to the maintenance of voice quality and reduces morbidity. It also excludes its main differential diagnosis, i.e., squamous cell carcinoma, which has a guarded prognosis.