OBJECTIVE: To report a case of Mycobacteriosis in the larynx in a patient treated at the Otolaryngology and Cervico-Facial Surgery at the Regional Hospital of Presidente Prudente in December of 2011. CASE REPORT: MR, 63 years old, male, born and resident in Santo Anastacio - SP, started 7 months before admission, odynophagia, progressive dysphonia, appetite loss, malaise and weight loss (10 kg), no cough, fever, dyspnea or other symptoms. Treatment was started with an infectious disease to Paracoccidioidomycosis, without improvement. At Fibronasolaryngoscopy (October 10th of 2011) showed exophytic lesion in the left vocal cord with apparent extension to supraglottis and larynx fixation. On November 24th of 2011 a biopsy was performed and material submitted to the Laboratory of Pathology, UNIFESP, whose results showed chronic granulomatosis process epithelioid, non-caseificant with ulceration and scattered foci of suppuration. We performed polymerase chain reaction (PCR), which confirmed Mycobacteriosis of the larynx. On December 14th of 2011 the patient admitted for treatment and research of co morbidities. Negative HIV serology. New Fibronasolaryngoscopy (December 22th of 2011) framework showed diffuse supraglottic laryngitis. Treatment was started with Coxip 4 and, after clearing of clinical symptoms, the patient was discharged. Remains in regular monitoring with Infectious Diseases, with gradual improvement. CONCLUSION: The atypical mycobacterium are a group of diseases caused by mycobacterium with well defined characteristics in terms of biochemical, immunological and pathogenic. Because it is insidious clinical findings, one should always take this hypothesis as a target for research in the granulomatosis lesions.