Background: Absence epilepsy is a common epilepsy syndrome in children. This can have a negative impact on the cognitive abilities of preschool and school-age children. The objective was to study in the Guinean context, the epidemiological, clinical, electrophysiological, therapeutic and evolutionary aspects of this syndrome. Methods: The study included all children diagnosed with absence epilepsy based on evidence obtained from history, clinical, and electroencephalogram. Results: The cohort was made up of 41 girls and 28 boys with a sex ratio (F/M) equal to 1.46. The mean age was 8 ± 2 years with extremes of 2 and 14 years. The simple absences were observed in 42.02% of cases. The components : tonic was associated in 11.59%, clonic in 10.14%, atonic in 13.04%, automatisms in 15.94% and vegetative in 7.25%. EEG was typical in 75.36%. As monotherapy, sodium valproate was used in 92.75% and ethosuximide in 2.9%. The evolution was marked by a remission of seizures in 85.51%. During follow-up, the appearance of tonic-clonic convulsions was noted in 4.3%, myoclonus in 2.9%, a combination of myoclonus and tonic-clonic convulsions noted in 4.3%. Conclusions: Effective and efficient collaboration between stakeholders is essential for the best overall management of this syndrome with serious cognitive repercussions in children.