Pulmonary Epithelioid Hemangioendothelioma (PEH) is one rather rare low-grade malignant tumor. Owing to its relatively low incidence, standard treatments on PEH are relatively less. A 52 y old male PEH patient treated with endostatin had received outstanding clinical outcome, considering his brain metastases and pleural effusion were both negative effects upon prognosis. Patient’s pathological characteristics, iconographic characteristics would play an essential role on his response to clinical treatment and prognosis. So far, standard treatments upon PEH are still not available, and the inhibition of tumor growth can be achieved through inhibition of tumor angiogenesis and interrupting cellular nutritious supply.