Pancreatic tuberculosis (TB) is an uncommon form of extrapulmonary TB and represents a diagnostic challenge for physicians. Pancreatic TB presents with nonspecific signs and symptoms and may mimic malignancy. However, pancreatic TB rarely occurs in children. Here, we present a case of a 5-year-old girl with pancreatic TB and markedly elevated serum cancer antigen- (CA-) 125 levels, thus raising the suspicion of malignancy, but positivity for Mycobacterium tuberculosis DNA was noted. The patient recovered after being administered standard antitubercular therapy for one year. This case suggests that clinicians should have a heightened suspicion of pancreatic TB when faced with pancreatic lesions despite the fact that increased CA-125 may indicate malignancy. Laparoscopy combined with peritoneal biopsy and polymerase chain reaction (PCR) may provide a new method to confirm the diagnosis.