Objective <p>To assess the long-term outcomes of surgically treated patients with ganglioglioma (GG).</p> Methods <p>This is a cross-sectional study of 70 patients submitted to surgery from 1997 to 2023 in our institution, with diagnosis of GG graded based on WHO 2021 criteria; At the same time, we reviewed the literature, gathering the most important papers, on some key points that impact the natural history of GGs, such as: location, surgical results, control of epilepsy (main symptom) and adjuvant treatments such as radiotherapy.</p> Results <p>The present series of our institution comprised 70 patients (43 males and 27 females) with the mean age of 10.12 ± 10.36 years old at the time of onset of symptoms; vast majority with grade 1 (G1) GG (97%). Seizure freedom (Engel I and II) was achieved in 79% of patients. The parameter anti-epileptic drug (AED) monotherapy had a significant impact on epilepsy control (<i>p</i> = 0.048). The literature revision of 1,089 GG cases confirmed tumor recurrence occurred in 14% and mortality rate was 7%. Seizure control was reported in 89% (Engel I and II), and adjuvant radiotherapy (RT) did not impacted in tumor recurrence that was 31% among those treated with RT and 23% in patients treated with surgery alone.</p> Conclusion <p>Epidemiological points were confirmed both in our review and in our case series: GG was more frequent in men, in the first two decades of life with seizures as the most frequent symptom. Surgical resection is essential, but in some locations, such as other tumors, complete resection is not possible, such as the brainstem and cerebellum, impacting the final result; epilepsy surgery led to better seizure control for those in anti-epileptic monotherapy. The impact of radiotherapy on natural history needs more research to reach a more precise conclusion.</p>