Untreated hyperammonemia can cause irreversible neurologic damage, coma, or death. A high level of clinical suspicion is necessary to quickly recognize and implement emergency interventions for hyperammonemia in the acute presentation of urea cycle disorders (UCDs). Pamela M. Peeke, MD, MPH, FACP, FACSM, is joined by Anna-Kaisa Niemi, MD, PhD, and a patient who was hospitalized for hyperammonemia at age 8 years to discuss the importance of improved recognition of hyperammonemia and awareness of the underlying causes, such as UCDs. Dr. Niemi is a neonatologist and associate professor at Rady Children’s Hospital in San Diego, California, USA. This podcast is sponsored by Horizon Therapeutics.