Dendriform pulmonary ossification (DPO) is a rare condition defined as disseminated, widespread heterotopic bone formation within the lungs. This condition is associated with restrictive pulmonary disease, such as interstitial pneumonia or fibrosis. The clinical features and pathophysiologic mechanism of DPO remain unclear, however. We report a case of a 66-year-old man with idiopathic pulmonary fibrosis accompanied by DPO who was treated with a double-lung transplant. His postoperative course was uneventful without recurrence of DPO.