A 40-year-old man with prolonged constitutional symptoms and clinical evidence of pancreatitis and biliary tract obstruction underwent exploratory laparotomy. Intraoperative liver and pancreatic biopsies revealed acid-fast bacilli. Mycobacterium tuberculosis subsequently grew from both sputum and urine cultures. The patient responded well to antituberculosis therapy, although 8 months later, he returned with acquired immunodeficiency syndrome (AIDS) and died of large cell lymphoma 1.5 years later. A review of the literature showed that most similar cases of pancreatic tuberculosis were diagnosed only at postmortem examination. A high index of suspicion and attention to special stains are warranted for diagnosis of this frequently fatal, but potentially curable, disease.