A unique case of severe, multiple, microbiologically-confirmed pulmonary Mycobacterium avium-intracellulare lesions occurred in a female with decompensated liver cirrhosis, and went to cure after around 2 y despite the impossibility to deliver an effective antimicrobial chemotherapy, owing to the patient's intolerance. From an extensive literature review, we underline a possible mechanism prompting mycobacterial disease during advanced liver disease, while we retrieved only 2 described cases of possible spontaneous resolution of Mycobacterium terrae lung disease, although both were reported in patients without end-organ liver disease, who received a specific combined chemotherapy for an appreciable time period. On the other hand, while decompensated liver cirrhosis is more frequently complicated by a peritoneal localization of bacteria and very infrequently mycobacteria, the reported case represents the first severe pulmonary localization of multiple lesions due to Mycobacterium avium-intracellulare. Moreover, this extraordinary episode resolved spontaneously within the 2-y follow-up, as documented by bronchoalveolar lavage, culture, high-resolution CT scans, and scintigraphic examination.