Tuberculosis cutis orificialis is a rare form of cutaneous tuberculosis. The article presents 5 clinical cases of tuberculosis cutis orificialis of the oral cavity and lips that were described in publications. We present our own clinical case of successful treatment of tuberculosis cutis orificialis of the oral cavity, red border of the lips and skin of the lower lip against the background of multiple drug resistant pulmonary tuberculosis in a HIV positive patient. The case was especially difficult due to a long diagnostic search (10 months), severe immunosuppression, and drug resistance of Mycobacterium tuberculosis.