Idiopathic pulmonary fibrosis (IPF) is an uncommon disease with a poor prognosis. Recently, two medications were approved to slow progression of disease. However, due to the limited treatment options available, patients and their families often look to experimental and off-label treatments for support. One such option is stem cell therapy, which can be delivered in a variety of ways. Stem cells are being utilized both in and out of the context of closely monitored clinical trials. However, there is little evidence regarding the safety and efficacy of stem cell therapy for patients with IPF. Here we describe a case of a patient who received intravenous and nebulized stem cell therapy, which was followed by an acute exacerbation of his IPF. The exacerbation was successfully treated with corticosteroids and supportive care.