iarjaccIARJACCIARJACC2709-1880https://doi.org/10.47310/iarjacc,2021.v02i01.08Klippel Feil Syndrome: A challenge to the AnaesthesiologistsDivyaChoudharyDheerajBhandariSuchetaTidkeNikhilYadavPostgraduate student in Department of Anesthesia, MGIMS, SewagramProfessor in department of Anesthesia, MGIMS, SewagramProfessor and Head of department of Anesthesia, MGIMS, SevagramAssociate Professor in department of Anesthesia, GIMS, Sevagram.Klippel-Feil syndrome (KFS) is a rare syndrome characterized by a classic triad comprising a short neck, a low posterior hairline, and limited neck mobility due to fused cervical vertebrae. We hereby report anesthetic management for pyeloplasty of KFS patient with micrognathia and restricted mouth opening. A careful preoperative evaluation to exclude other anomalies associated with   KFS was done. The patient had restricted mouth opening, retrognathia, and limited cervical mobility due to cervical vertebral fusion, with sprengels deformity, aortic arch abnormality, sensory neuronal hearing loss and urologically pelviuretric obstruction on both sides. As difficult intubation was predicted, video laryngoscopy was our first choice for gaining control of the patient’s airway. In the operating room, tracheotomy equipment was kept ready if a perioperative surgical airway control was required. Surgery was uneventful. Careful preoperative examination and preparation for difficult airway management are important for KFS patients with micrognathia.