We describe a child with Panayiotopoulos syndrome (PS) who presented with autonomic status epilepticus and developed respiratory arrest requiring intubation and mechanical ventilation. Because of that life-threatening episode and the risk of developing a similar event in subsequent seizures, we decided to initiate our patient on AED treatment. Such life-threatening complications were previously reported in only four children with PS. Although PS is considered to be a benign childhood epilepsy syndrome usually not requiring treatment with antiepileptic drugs, our case and the small number of similar cases in the literature show it is important to realize that it can rarely be associated with life-threatening complications. It is our opinion that children with PS who develop an episode of autonomic status epilepticus and those living in remote areas with no quick access to emergency departments should be initiated on AED therapy to minimize the risk of experiencing a subsequent potentially fatal seizure. We further suggest that the use of benzodiazepines in this syndrome should only be administered during the early stage of the seizure, since administration of this class of drugs during an established autonomic status epilepticus can result in further respiratory depression.