We report an extremely rare case of intrapulmonary lymphangioma. A 41-year-old woman was admitted to our hospital for complete medical evaluation of a pulmonary nodule on chest X-ray, which was found during an annual check-up. Chest radiograph showed a well-circumscribed spherical mass adjacent to the right border of the heart in the right lower lung field. Although a cystic tumor of the lung was suspected based on computed tomogram and magnetic resonance imaging findings, a histologic diagnosis could not be made on transbronchial biopsy. In addition, the nodule had increased in size compared with a chest X-ray taken 1 year previously. Enucleation was performed. Histological examination revealed numerous cysts with thin walls lined by a single layer of cells in which immunoreactivity to von Willebrand factor was weakly positive. Intrapulmonary lymphangioma was subsequently diagnosed.