Introduction: Epilepsy is a cause of disability and morbidity in children and adults. Dravet Syndrome (DS) is an epileptic encephalopathy that develops early in childhood, most often associated with a loss-of-function mutation in the SCN1A gene. Currently, around 30 anticonvulsants are available for use, most of which have been recently approved. Anticonvulsant therapy results in freedom from seizures in 70% of pediatric patients. The choice of treatment must be made by relating the patient's characteristics, side effects of the antiepileptic drug, social and socioeconomic factors. The difficulty in treating refractory epilepsies indicates the importance of developing new drugs. Objectives: The research aims to evaluate publications on the efficiency of using cannabidiol associated with standard antiepileptic treatment in DS and the adverse effects of this intervention. Methods: The research consists of a literature review carried out by searching for publications in the PubMed, Scielo and Cochrane Library databases between 2018 and 2024, with the keywords “Dravet syndrome” and “Dravet syndrome and treatment and cannabidiol”. The inclusion criteria were original articles, systematic reviews, meta-analyses and double randomized clinical trials available free of charge in Portuguese, English or Spanish. Exclusion criteria were duplicate articles, articles that did not evaluate the use of cannabidiol associated with standard epilepsy treatment in Dravet syndrome. Results: Among the 10 studies analyzed, all showed positive results regarding the standard treatment of epilepsy associated with cannabidiol, reducing the number and frequency of seizures in patients. The treatment showed acceptable safety, however, the incidence of adverse effects was sensitive. The adverse effects indicated in 100% of the studies were elevation of liver enzymes (mainly in patients who used sodium valproate associated with treatment) and drowsiness. Furthermore, the effects of decreased appetite (90%), diarrhea (70%), pyrexia (50%), vomiting (40%) and gastrointestinal discomfort (10%) were common. Conclusion: The difficulty in treating epilepsy in patients with Dravet syndrome indicates the need for a combination of different drugs. Cannabidiol can be considered a safe and effective option to combine with treatment, as it reduces the number and frequency of epileptic seizures. However, it presents important adverse effects such as decreased appetite, drowsiness, diarrhea, pyrexia, vomiting and elevation of liver enzymes, which is more pronounced in patients who use sodium valproate associated with treatment. Therefore, the complexity of the treatment and the scarcity of studies highlight the importance of more research to better evaluate adverse effects and drug interactions.