The presence of lone atrial fibrillation presupposes the absence of structural heart disease, hypertension, or other known predisposing factors. However, several recent clinical and experimental data provide novel insights into the pathogenesis of lone atrial fibrillation. In addition, modern diagnostic techniques often reveal some previously unappreciated abnormalities. Therefore, the increasing recognition of potential causes and links sets the base for a more complete elucidation of its etiology in the near future. This concise review article discusses the contemporary advances in the understanding of this form of atrial fibrillation.