Idiopathic pulmonary fibrosis (IPF) is a rare disease of unknown etiology and is usuallyassociated with a poor prognosis. Up to the present, less than 50 cases of IPF in childrenhave been reported in the English literature, and no case has ever been reported fromTaiwan. Herein we report on a 2-year-old boy with IPF presenting with a rapid onset of dyspneafollowed by respiratory failure. The diagnosis of IPF was verified with an open lungbiopsy. Despite intravenous methylprednisolone pulse therapy and empiric nitric oxidetreatment, he expired on the 35th day after admission due to profound hypoxemia. A diagnosisof IPF should be included in the differential diagnosis for patients presenting withunexplained shortness of breath and pulmonary interstitial infiltrations.