Extramedullary manifestations of acute myeloid leukemia (AML), often referred to as myeloid sarcoma (MS), occur relatively commonly in children with newly diagnosed or relapsed AML and have been associated with certain French-American-British morphologies and gene/chromosomal rearrangements. The most common locations of MS include the skin, orbit, skeleton, central nervous system, skin, and gut. Pulmonary MS is uncommon in adults and is extremely rare in children. We report the case of a 19-year-old man with French-American-British M5 AML, who before bone marrow transplant, presented with fever, hypotension, and respiratory symptoms that were ultimately attributed to pulmonary MS.