In this report, we describe three cases of PEH. Case 1, a 61-year-old man, had nonspecific chest discomfort and the chest X-ray showed a solitary lung nodule. This nodule was diagnosed by an open lung biopsy. The pathologic findings including abundant necrosis, mitosis and hyperchromatic and pleomorphic nuclei, indicated a malignancy. The electron microscopic study showed Weibel-Palade bodies and the immunohistochemical stain for CD31 showed a positive reaction in the tumor cells, and linear staining along the vascular lumina. Case 2, a 34-year-old man, was admitted for an evaluation of multiple small nodules, incidentally detected a screening chest X-ray. The nodules were diagnosed by a immunohistochemical stain for the factor VIII-related antigen. Case 3, a 34-year-old woman, complained of left pleuritic chest pain. A simple chest film and the chest CT scan revealed multiple bilateral nodules and a left pleural effusion. An immunohistochemical stain for the factor VIII-related antigen was used to diagnose the nodules. Forth-one months after the diagnosis, she died of pulmonary insufficiency.