Stiripentol (STP), an antiseizure medication used for Dravet syndrome (DS), has multiple mechanisms of action (MoAs). It enhances GABAergic transmission by inhibiting GABA reuptake, blocking GABA transaminase, and acting as a positive allosteric modulator of GABA_A receptors, particularly those containing α3 and δ subunits. STP also blocks voltage-gated sodium and T-type calcium channels, and regulates glucose metabolism and lactate dehydrogenase (LDH). It inhibits several cytochrome P450 enzymes, enhancing the anticonvulsant effects of other antiseizure medications. These MoAs contribute to its efficacy in treating DS and may have potential in other neurological and non-neurological diseases. STP is effective in reducing seizure frequency and preventing status epilepticus (SE) in DS patients. It also shows neuroprotective effects by modulating ion channels and energy metabolism. STP's interactions with other anticonvulsants, such as clobazam, can influence their metabolism and efficacy. Additionally, STP has shown potential in treating other conditions like primary hyperoxaluria and glioblastoma by inhibiting LDH and reducing lactate production. Overall, STP's multifaceted MoAs suggest its broad therapeutic potential beyond epilepsy.Stiripentol (STP), an antiseizure medication used for Dravet syndrome (DS), has multiple mechanisms of action (MoAs). It enhances GABAergic transmission by inhibiting GABA reuptake, blocking GABA transaminase, and acting as a positive allosteric modulator of GABA_A receptors, particularly those containing α3 and δ subunits. STP also blocks voltage-gated sodium and T-type calcium channels, and regulates glucose metabolism and lactate dehydrogenase (LDH). It inhibits several cytochrome P450 enzymes, enhancing the anticonvulsant effects of other antiseizure medications. These MoAs contribute to its efficacy in treating DS and may have potential in other neurological and non-neurological diseases. STP is effective in reducing seizure frequency and preventing status epilepticus (SE) in DS patients. It also shows neuroprotective effects by modulating ion channels and energy metabolism. STP's interactions with other anticonvulsants, such as clobazam, can influence their metabolism and efficacy. Additionally, STP has shown potential in treating other conditions like primary hyperoxaluria and glioblastoma by inhibiting LDH and reducing lactate production. Overall, STP's multifaceted MoAs suggest its broad therapeutic potential beyond epilepsy.