A patient with a 5-month history of pericardial effusion and unsuccessful steroid treatment was found at pericardial exploration to have thickened pericardium adhered to the heart. After anterior pericardiectomy, histologic examination revealed severe granulomatous pericarditis resulting from infection with Mycobacterium tuberculosis. Despite the pericardiectomy and antituberculous therapy, the patient continued to have symptoms, including bilateral pleural effusions, 4 days after discharge from the hospital. After a second exploration (after echocardiography revealed decreased left ventricular function and hemodynamic findings showed persistent constriction), anterior and posterior cardiectomy was deemed necessary. Pleural effusion did not recur, and the patient was discharged with antituberculous drug therapy. This rare extrapulmonary form of tuberculosis can have an insidious or sudden onset, and diagnosis is complicated by false-negative tuberculin tests, nonspecific radiographic and echocardiographic findings, and time-consuming bacteriologic culture. Previous high mortality with the disease has been decreased to approximately 40% by the advent of antituberculous drug therapy. The still significant mortality reflects the difficulty in early diagnosis and the serious effects of pericardial effusion and constriction.