Background Neonatal seizures are often refractory to treatment with initial antiseizure medications. Clinicians turn to agents such as levetiracetam despite the paucity of published data regarding its safety, tolerability, or efficacy in the neonatal population. Patient presentation We describe a neonate who developed anaphylactic shock developing after receiving intravenous levetiracetam. Results This is the first neonate to develop anaphylactic shock due to intravenous administration of levetiracetam. Conclusion Clinicians should be aware of this potentially fatal adverse effect occurring with intravenous levetiracetam in newborns.