Gemcitabine is a nucleoside analogue used for treatment of multiple cancers. Recent reports describe small to medium vessel vasculitis as a rare complication of therapy. We report a case of significant large vessel vasculitis presenting as fever of unknown origin in a patient treated adjuvantly with docetaxel and gemcitabine for uterine leiomyosarcoma.The patient presented after her first and second exposure to chemotherapy with fever of unknown origin and worsening subsequent chest and neck discomfort. Extensive workup revealed radiographic and laboratory evidence of aortic vasculitis. We initiated treatment with high-dose corticosteroids, and the patient's condition resolved without complication over the next several weeks.To our knowledge, this represents the first case of gemcitabine-induced large vessel vasculitis which presented in our case as fever of unknown origin. Awareness of this potential complication is essential as discontinuing the inciting drug and initiating corticosteroid treatment can be life-saving.