Childhood absence epilepsy (CAE) is a common pediatric epilepsy syndrome accounting for 10% of all pediatric epilepsies. The aim of this review is to provide an updated overview of this epilepsy syndrome to pediatricians. Most of the patients can be initially managed in private practice or in general pediatric settings. Absence seizures are the only seizure type observed at the time of diagnosis in these patients. An electroencephalogram recording and a clinical evaluation lead to the diagnosis. The underlying mechanisms are not yet fully understood. CAE is considered a self-limited epilepsy syndrome since most of the patients will become seizure free. Only a few patients (5-10%) have resistant CAE. However, CAE, as well as any pediatric epilepsy syndrome, should not be considered a "benign" epilepsy. Attention deficit is observed in about one third of the patients. There is also an increased risk of academic difficulties related to specific cognitive disorders. Therefore, the early detection of children at risk of developing neuropsychological problems can be helpful for preventing school underachievement and poor psychosocial outcome. Recently, several studies including a large randomized controlled trial indicated that ethosuximide should be considered as a first-line treatment and valproate as a second-line treatment. Cognitive side effects should be an important factor in the selection of the antiepileptic drug and should be specifically assessed during the follow-up. This review concludes by discussing the criteria that might lead to referring the patient to a specialist.