A 48-year-old immunocompetent man without known exposure to tuberculosis had a > 10-year history of recurrent skin lesions. Cutaneous tuberculosis without any current or past history of pulmonary tuberculosis was diagnosed. Culture of biopsy specimens showed the organism to be resistant to multiple first-line and second-line agents. The patient had a broad, vigorous CD4-specific immune response against multiple tuberculosis antigens. This case is the first report of cutaneous extensively drug-resistant tuberculosis.