Bronchiolitis obliterans (BO) is a significant life-threatening complication that occurs after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and it is associated with increased morbidity and mortality. BO responds poorly to corticosteroids or immunosuppressants, and there are currently no established treatment approaches. We herein describe a patient with biopsy-proven BO after allo-HSCT who was successfully treated with tamibarotene, a novel synthetic retinobenzoic acid. Tamibarotene led to a dramatic improvement in lung function as well as cutaneous manifestations of chronic graft-vs-host disease. A large prospective clinical trial is therefore warranted to confirm the efficacy of tamibarotene in BO.