Transcatheter aortic valve replacement (TAVR) is the best treatment for severe aortic stenosis with high surgical risk, and low body weight significantly increased the risk of surgery and postoperative all-cause mortality. A case of elderly female diagnosed with severe aortic valve stenosis and extremely low body weight was presented in this article. Additionally, horizocardia and low located coronary orifice were also found in this patient, which markedly increased the risk and complexity of the TAVR procedure. During the operation, circulatory collapse occurred, and prosthetic valve was quickly released under emergency cardiopulmonary resuscitation. The operation was successful and the patient’s vital signs recovered soon. The follow-up showed that the patient was in good condition.