Frontometaphyseal dysplasia (FMD) is a rare X-linked hereditary disorder. Skeletal deformities are common manifestations. Patients usually undergo surgeries early in life. The anesthetic management of a patient with FMD is presented in the current case report.

A 6 years old female patient, weighing 20kg and height 120cm, admitted to hospital and scheduled for genu recurvatum correction. Her eyebrows were prominent, she had defects in teeth, proptosis, micrognathia, pectus carinatus, genu recurvatum and bowing tibia, and 2o over 6o murmur. Right tympanic membrane was perforated. Induction and maintenance of anesthesia was uneventful.

FMD patients are candidates for difficult airway management and anesthesiologists taking care of a patient with FMD must be prepared for an anticipated difficult airway. For a safe anesthetic management in FMD, a careful and detailed preoperative visit and consultation with related departments, strict monitorization at the operation room and being prepared for an anticipated difficult airway is essential.