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Pulmonary epithelioid hemangioendothelioma is a rare pulmonary neoplasm. No reliably effective systemic chemotherapy for this disease entity has been established yet. Surgery is usually applied for patients with a small number of lesions limited to one lung, while feasibility/efficacy/safety of this modality is controversial for patients with bilateral multiple lung lesions.  Presence of cases showing rapid aggravation and those showing very gradual progression makes it difficult to evaluate validity of surgery as a suitable therapeutic approach. Herein, we present a summary on pulmonary epithelioid hemangioendothelioma and discuss the role of surgery in the treatment of this condition.