Darvet syndrome is rare, drug-resistant epilepsy that begins in the first year of life in an otherwise healthy infant. It usually presents with a prolonged seizure with fever that affects one side of the body. Most cases are due to severe SCN1A gene mutation. Darvet syndrome is rare, drug-resistant epilepsy that begins in the first year of life in an otherwise healthy infant. It usually presents with a prolonged seizure with fever that affects one side of the body. Most cases are due to severe SCN1A gene mutations. Most children develop varying degrees of developmental disability. Treatment involves finding the best combination of medicines to treat seizures and prevent seizure emergencies. Dietary therapy and vagus nerve stimulation can also be helpful. Two new medications have been approved by the FDA specifically for Darvet syndrome. These include Epidiolex (cannabadiol, CBD) oral solution approved in June 2018, and Stiripentol approved in August 2018.A multidisciplinary team and early developmental assessment are needed to address the many ways Darvet syndrome can affect a child and their family.