BackgroundThe aim of this study was to investigate the frequency and characteristics of pediatric epilepsy in the geographic isolate of Sardinia island and to calculate the prevalence of active epilepsy. MethodsThe study was retrospective, observational and involved a systematic review of medical records and computerized archives containing all clinical and EEG recordings of patients with epilepsy referred to the regional structures that could have followed patients with epilepsy in South Sardinia, during the period 2003–2021. ResultsThe study population included 112,912 children and adolescents (age ≤ 18 years). 618 children and adolescents (women 42.4 %) were identified. Family history of epilepsy was reported in 153 (26.1 %). Etiology was genetic in 64.5 % and structural in 26.7 % subjects. Focal seizures were reported in 51.6 % of subjects, followed by 34.7 % with generalized seizures and 10.6 % of patients experienced both type of seizures.A total of 301 subjects with active epilepsy in 2019 were identified resulting in a prevalence of 2.67 per 1000 (95 % CI 2.37–2.97). Prevalence in the age class 5–14 years was 4.21 per 1000 (95 % CI 3.72–4.76). ConclusionCompared to the previous studies in distinct geographic isolates, the present study showed a significantly low prevalence rate of active epilepsy; a high percentage of focal seizures and genetic etiology.