The case of a 9-year-old girl with a Mycobacterium tuberculosis inflammatory myofibroblastic tumor (IMT) of the left lobe of the liver is reported. The tumor was surgically excised and had histological features diagnostic of IMT, a positive Ziehl-Nielsen staining for acid-fast bacilli and a positive polymerase chain reaction for Mycobacterium tuberculosis. Surgical excision of the tumor followed by anti-tuberculosis treatment for 9 months resulted in full recovery. The patient had no apparent immune disorder, and there was no evidence of extrahepatic tuberculosis. These findings make this case exceptional because IMTs, due mostly to atypical mycobacteria, have been described only in immunocompromised patients.