Aims This study sought to investigate electrocardiographic characteristics, electrophysiological features, and radiofrequency ablation in patients with focal atrial tachycardia (AT) originating from the septal mitral annulus. Methods and results In 13 patients with AT originating from the septal mitral annulus, activation mapping was performed to identify the earliest activation site. Successful ablation was performed through either a transseptal (n = 12) or a retrograde aortic approach (n = 1). As confirmed by electrogram recordings, fluoroscopy, and three-dimensional (3D) mapping, successful ablation sites were located in the anterior paraseptal, mid-to anteroseptal, and posterior septal mitral annulus in eight, three, and two patients, respectively. Foci for all locations demonstrated a negative/positive appearance in lead V1. Mapping in the right atrium demonstrated that the earliest right atrial activation was near the septum (His-bundle region or proximal coronary sinus). The electrograms at the successful ablation sites were fractionated in 9 patients, and presented with an atrial: ventricular ratio of <1 in all 13 patients. There were no complications in any patients and long-term success was achieved in 12 of 13 patients during the 23 +/- 6 months following ablation. Conclusion The area surrounding the septal mitral annulus, most commonly the anterior paraseptal, is an unusual, but important site of origin for focal AT, which is associated with a distinctive P-wave morphology and atrial endocardial activation sequence. Radiofrequency ablation of AT originating from the septal mitral annulus, through either a transseptal or a retrograde aortic approach appears to be safe and effective.