The purpose of this work was to expound on the postulated pathological mechanisms through which pheochromocytoma/paraganglioma (PPGL) can cause seizures by conducting a comprehensive review of ten cases and several pathogenic mechanisms. The goal was to enhance awareness amongst doctors and researchers about patients with PPGL presenting with seizures. This would help decrease the risk of misdiagnosis and mismanagement in future clinics. Additionally, this review was written with the purpose to attract more attention to etiological explorations, particularly concerning rare causes of seizures, which is consistent with the idea that League Against Epilepsy (ILAE) has emphasized in the new version of the ILAE position paper published in 2017. It is of great importance to keep in mind the fact that seizures can constitute an atypical presentation of PPGL and to establish early diagnosis and accurate cure for these patients, especially in the presence of paroxysmal hypertension or other suggestive symptoms of PPGL.