Pulmonary metastasis from carcinoma of the papilla of Vater is considered to be a late event, and patients can be treated with radiotherapy, chemotherapy, or palliative surgery. However, surgical treatment of an isolated lung metastasis has not been reported. We present a surgical case of solitary pulmonary metastasis from carcinoma of the papilla of Vater. A 51-year-old man underwent pylorus-preserving pancreaticoduodenectomy for Vater carcinoma. During follow-up, chest computed tomography revealed a nodular shadow in the right lung. The pathological examination demonstrated the appearance of the pulmonary tumor resembled that of the previously resected Vater carcinoma, and both tumors showed similar immunostaining properties, leading to the pathological diagnosis of pulmonary metastasis from carcinoma of the papilla of Vater. Isolated pulmonary metastasis from carcinoma of the papilla of Vater is extremely rare, but surgery could be the treatment of choice.