Abstract Phenylketonuria is a rare disorder that increases the levels of phenylalanine in the blood. As there are scant articles about anesthesia management in phenylketonuria patients, this encouraged us to report a short-time anesthesia management of a child with phenylketonuria for bone fracture. The anesthesia was induced with intravenous ketamine and midazolam. During procedure, he received 100% oxygen via a face mask throughout spontaneous breathing. The operation was uneventful, and he was completely awakened in the recovery room. This report emphasizes that in some situations, the combination of midazolam with ketamine could be used safely for short time anesthesia in phenylketonuria patients.