Background and Objective: Low-grade glioma (LGG) is a common type of brain glioma. It frequently presents with epilepsy, which is often the only symptom of the LGG. Given the clear differentiation between neural and glial cells, the overall prognosis for LGG is favorable. Therefore, in LGG-related epilepsy, seizure control is more critical than tumor resection. If the seizures are not controlled, they can develop into drug-resistant epilepsy (DRE), adversely affecting patient development, quality of life, and psychology. This review focuses on the epidemiology and diagnosis of LGG-related epilepsy and discusses several influencing factors and surgical strategies. Methods: We searched through current literature, focusing on articles related to epidemiology, diagnosis, influencing factors, and surgical strategies for LGG-related epilepsy. Key Content and Findings: LGG-related epilepsy is usually DRE, which is refractory to AEDs. In general, LGG-related epilepsy may occur in up to 90% of LGG cases. Regular anti-epileptic drug (AED) therapy is necessary when LGG-related epilepsy is diagnosed, irrespective of etiology. AED selection will depend on seizure type, patient age, underlying diseases, among other factors. If LGG-related epilepsy is diagnosed, the surgical goal is maximal safe resection to render the patient seizure-free. Preoperative evaluation should be for "epilepsy surgery" rather than "tumor surgery". Radiotherapy and chemotherapy may improve the suizer control after surgery. As LGG is slow-growing, the overall prognosis is excellent, with a median survival period of 5-10 years, which can extend up to 20 years. Conclusions: If LGG-related epilepsy is diagnosed, any preoperative evaluation should be for "epilepsy surgery" rather than "tumor surgery". The aim of surgical treatment is a maximal safe resection to render the patient seizure-free. The gross total resection of the LGG and surrounding epileptogenic zones is the primary positive prognostic factor for seizure control. Radiotherapy and chemotherapy both play a role in seizure control in cases with residual LGG.