Metabolic Emergency in Flight

•Children with undiagnosed inborn errors of metabolism often present in metabolic crisis.•Metabolic crises can result in significant metabolic derangements, altered mental status, coma, and death if untreated.•Expeditious transport to definitive pediatric care can treat and prevent severe neurologic sequelae and death of these undifferentiated patients.•Pediatric specialty transport teams help focus treatment considerations on pediatric-specific pathologies while awaiting definitive diagnostics.•Transport teams without pediatric providers should be aware of these potential disorders that do not respond typically to standard resuscitative measures.