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Abstract Background The case of aortic valve stenosis complicated with lung cancer have compelled cardiovascular surgeons to make challenging. We report the first successful short-term outcomes of one-stage minimally invasive aortic valve replacement and video-assisted thoracoscopic surgery lobectomy through right mini-thoracotomy in a patient with synchronous bicuspid severe aortic valve stenosis which was unsuitable for transcatheter aortic valve implantation and right lung cancer. Case presentation A 76-year-old man with severe aortic valve stenosis was diagnosed with lung cancer of the right upper lobe with stage IA2. Considering the potential risk of tumor metastasis, a one-stage surgical therapy for right lung cancer and type 0 bicuspid aortic valve stenosis was required; however, transcatheter aortic valve implantation was unsuitable due to a bicuspid aortic valve with severe calcification. Therefore, concomitant minimally invasive aortic valve replacement and lobectomy via right mini-thoracotomy were performed. The postoperative course was uneventful. Conclusion Concomitant aortic valve replacement and right lobectomy via right mini-thoracotomy may reduce surgical invasiveness, leading to early recovery. This surgical strategy is a useful option, particularly for patients with aortic valve stenosis complicated with right lung cancer.