Primary pulmonary EWS/PNET(PPES) is extremely rare and is associated with a poor prognosis. Tumor angiogenesis plays an important role in tumor, so it has become a hot topic in molecular targeted therapy. Anlotinib is a new oral small molecular multi-targeted receptor tyrosine kinase (RTK) inhibitor. This report describes a 20 year-old man with PPES. After 4 neoadjuvant chemotherapy cycles (VACwith alternating IE) combined with anlotinib, the left total pneumonectomy was performed. Then maintenance anlotinib monotherapy was continued, no sign of recurrence to date as an outcome. To our knowledge, this is the first demonstration of anlotinib combined with neoadjuvant chemotherapy efficacy in PPES.