Background: Focal atrial tachycardia (AT) originating from the right atrial appendage (RAA), often persistent and refractory, is clinically rare in pregnant woman, and the therapy is much more challenging. We report that a pregnant woman presented with hypotension due to persistent and refractory atrial tachycardia and was successfully cured by a multidisciplinary treatment (MDT) approach, consisting of a combination of zero-fluoroscopy mapping and thoracoscopic atrial appendectomy. We also carried out a literature review of this topic. Methods and Results: A 26-year-old woman in pregnancy at 21 weeks presented with severe palpitation and hypotension due to persistent rapid supraventricular tachycardia (SVT). Since adenosine triphosphate could not terminate the tachycardia, a catheter ablation procedure was planned and finally canceled when the zero-fluoroscopy mapping using Carto 3(TM) system revealed an atrial tachycardia originating from the RAA. Thoracoscopic RAA ectomy was recommended after multidisciplinary consultation and successfully performed without fluoroscopy. Ensite(TM) velocity mapping system was used for accurately locating the origin of the arrhythmia during ectomy. The woman finally produced a healthy baby during follow-up. Conclusions: Focal AT originating from appendage in pregnant patients can be persistent, refractory, and life-threatening; traditional strategies, such as medicine or catheter ablation, are limited in this situation. MDT measures, using a thoracoscopic ectomy and zero-fluoroscopy three-dimensional electroanatomical mapping technique, is minimally invasive and a promising strategy.