Two cases of children with breath-holding spells and documented prolonged asystole are reported. We discuss the different clinical and pathophysiological subgroups of this entity and the possible connection to neurocardiogenic syncope. For a certain defined subgroup of breath-holding spells, its classification as an early manifestation of neurocardiogenic or neurally mediated syncope would be more correct. This would have implications for further diagnostic as well as therapeutic procedures.