Primary multidrug-resistant Mycobacterium tuberculosis is an important problem in the United States. There is no report in formal literature of this pathogen in Brazilian patients. CASE REPORT--We report a case of ganglionar tuberculosis diagnosed by acid-fast smears in a male, HIV positive patient. Mode of acquisition of HIV was not determined. Treatment was started, and isoniazid, rifampicin and pyrazinamide were prescribed. The patient and his family reported strict adherence to therapy, but no improvement was observed. After 75 days, the patient was admitted in our hospital because of clinical worsening. Clinical features were the presence of large submandibular and axillar lymph nodes, respiratory insufficiency and complains of abdominal pain. He died six days after admission. Culture obtained from the ganglionar aspirate disclosed M. tuberculosis susceptible to ethambutol, but resistant to isoniazid, rifampicin, pyrazinamide, ethionamide and streptomycin. DISCUSSION--Although this was a case of extrapulmonary tuberculosis, there is a concern about multidrug-resistant tuberculosis, that has been poorly evaluated in Brazil. Since high lethality and intrahospital transmission have been reported, we discuss the need of performing culture and antibiogram in suspected cases, and the prevention of the spread of M. tuberculosis to patients and health-care workers through the strict adherence to the isolation practices.