Sweet's syndrome (SS) is an acute, febrile neutrophilic dermatosis that frequently presents with leukocytosis and erythematous plaques. Lesions show neutrophilic infiltration of the dermis and rarely other organs. We report the case of an adolescent male with chronic pancytopenia secondary to Fanconi anemia (FA) who presented with acute respiratory distress. Despite an exhaustive and ultimately unrevealing work-up, the diagnosis of pulmonary SS was not made until he developed characteristic cutaneous lesions 4 months later. Comprehensive review of pathological specimens revealed metachronous SS manifestations with infiltrates in lung parenchyma, dermis, and subcutis in this neutropenic patient. Pediatr Blood Cancer 2008;51:128–130. © 2008 Wiley-Liss, Inc.