Background: Dravet syndrome (DS) is a rare treatment-resistant epileptic encephalopathy, with a high mortality rate, resulting from mutations in the SCN1A gene (encoding the α1 subunit of the NaV1.1 channel). Studies show that cannabidiol is effective in reducing the frequency of seizures. Objective: Analyze the efficacy of cannabidiol for the treatment of refractory epilepsy in DS. Methods: In April 2021, the PubMed database was queried to search for the terms “Cannabidiol” AND “Dravet Syndrome”, with the filters: Clinical trial, Randomized controlled trial and the articles were limited to the last 5 years. With this, 8 articles were found, of which 3 were selected. Exclusion criteria: (1) articles that didn’t evaluate the effectiveness of treatment (2) review articles. Results: A randomized, double-blind study of 120 patients, which a dose of 20 mg/kg/day was used, found that cannabidiol use led to lower frequency of seizures, reducing from 12, 4 to 5.9 seizures per month; and 5% of participants were seizure-free. A similar reduction was documented by an open clinical trial, which there was a reduction of 39.5% in total seizure frequency and 2.9% were seizure-free. Additionally, another randomized, double-blind study reported a percentage reduction in seizure frequency of 48.7% for the group of patients given 10 mg/kg/day, and 45.7% for the group given 20 mg/kg/day. Conclusion: In DS patients, cannabidiol shows efficacy to reduce the frequency of seizures. However, more studies are needed to validate its application.