Background <p>This study aims to report a case of pulmonary epithelioid hemangioendothelioma (PEH) achieving pathological complete remission following neoadjuvant treatment, thereby enhancing clinical understanding of this rare disease and providing valuable treatment insights.</p> Case presentation <p>The patient’s imaging examination revealed a single large tumor on one side of the lung with lymph node metastasis. After treatment with albumin paclitaxel + carboplatin + bevacizumab, the tumor disappeared from the imaging findings, and postoperative pathology confirmed that the patient achieved pathological complete remission.</p> Conclusion <p>PEH is rare in clinical practice and is a type of malignant tumor without characteristic clinical manifestations, and the diagnosis relies on pathological and immunohistochemical examinations. Single or few lesions can be treated surgically, whereas those that cannot undergo surgery can be treated with chemotherapy and targeted therapy.</p>