From February/2023, the patient received entrectinib at a dose of 600 mg once daily. One month later after TRK-TKI, tumor markers returned to normal , left pleural tumor and right chest wall subcutaneous mass disappeared completely…We present the case of a patient with lung atypical carcinoid with NTRK gene fusion on re-biopsy after multiple line therapy failure of platinum-based chemotherapy , targeted therapy, even immunotherapy . Entrectinib seventh line of therapy, achieved complete response, which emphasizing the efficacy of this selective NTRK kinase inhibitor on this rare neuroendocrine tumor with NTRK fusion.