Acute respiratory distress syndrome (ARDS) is a serious complication of sepsis. Only a few cases of non-tuberculous mycobacteria complicated by ARDS have been discussed in the literature to date. Mycobacterium kansasii is the most pathogenic non-tuberculous mycobacterium affecting the lung. In the late stages of acquired immunodeficiency syndrome (AIDS), it may also present as disseminated disease. The clinical course is usually chronic, and the time to clinical diagnosis can sometimes be long, requiring a careful and meticulous search for the pathogen. To our knowledge, this is the first case report of disseminated M. kansasii infection complicated by ARDS and acute liver failure in a patient with end-stage AIDS.