Childhood absence epilepsy (CAE) is a common epilepsy syndrome characterized by absence seizures affecting young children and representing 18% of all diagnosed cases of epilepsy in school-age children. Absence seizures are classically very frequent during the day and each seizure lasts a short time, from about 10 to 20 seconds, it ends abruptly, and awareness and responsiveness are severely impaired. The typical EEG pattern in CAE is a bilateral, synchronous, and symmetrical discharge of complex spike-wave rhythms at 3 Hz (range of 2.5–4 Hz), with sudden onset and termination. CAE is genetically determined, the mode of inheritance and genes involved remain not fully clarified but the final outcome is the dysregulation of cortico-thalamic-cortical circuit that plays a crucial role in the pathophysiology of absence seizures. CAE may have an impact on patients’ lives in terms of negative consequences in neurocognitive and neuropsychological aspects that should always be considered during a global evaluation of a child with epilepsy.