Focal atrial tachycardia (AT) originating from the left atrial appendage (LAA) is one of the rare supraventricular tachycardias and is likely to cause arrhythmia-induced heart failure. Surgical treatment could be an alternative therapy because antiarrhythmic drugs and catheter ablation therapy to focal AT originating from the distal portion of the LAA is still challenging. We report a case of successful operation of minimally invasive thoracoscopic appendectomy in a patient with poor left ventricular (LV) function due to drug-resistant AT originating from the LAA for the first time. A 51-year-old female who had AT with a poor LV function suffered from congestive heart failure. We diagnosed the ongoing AT as focal AT that originated from the distal portion of LAA by electrophysiological examination. Total thoracoscopic stand-alone appendectomy was performed safely. AT was terminated and restored to sinus rhythm immediately after appendectomy. <Learning objective: Although catheter ablation has become a first-line treatment for almost all cardiac arrhythmias, it is difficult to achieve complete cure of atrial tachycardia (AT) originating from the distal portion of left atrial appendage (LAA) because there is AT recurrence and risk of cardiac perforation and ischemic stroke. Minimally invasive thoracoscopic appendectomy is curative and can be applied safely even in patients who have poor left ventricular function due to focal AT originating from the LAA.>