Tehran University of Medical Sciences Archives of Anesthesiology and Critical Care 2423-5849 6 3 2020 08 18 142 144 EN Vamshi Krishna Department of Anesthesiology, All India Institute of Medical Sciences Rishikesh, Rishikesh, India. Nishith Govil Department of Anesthesiology, All India Institute of Medical Sciences Rishikesh, Rishikesh, India. Vijay Adabala Department of Anesthesiology, All India Institute of Medical Sciences Rishikesh, Rishikesh, India. Jitender Chaturvedi Department of Neurosurgery, All India Institute of Medical Sciences Rishikesh, Rishikesh, India. 2020 01 07 2020 04 06 Meckel Gruber syndrome (MGS) is one of the rare autosomal recessive inherited disorder affecting several organ systems of the body. It is specifically characterised by occipital encephalocele, bilateral polycystic kidney, post axial polydactyly making the airway management difficult. We would like to share successful management of a case of Meckel Gruber syndrome with giant occipital-cervical encephalocele posted for excision. Difficult airway cart was kept ready prior to intubation. This includes laryngeal mask airway of appropriate size, high frequency jet ventilation, fibre-optic bronchoscope, a cricothyroid cannula and preparations for tracheostomy. Anaesthesia to paediatric age group poses many challenges to anaesthesiologist and that for congenital anomalies add to it. Working knowledge of these kind of syndromes and specific considerations for the same should be kept in mind when assessing a case of occipital encephalocele leading to more effective and appropriate management of cases. https://aacc.tums.ac.ir/index.php/aacc/article/view/274 https://aacc.tums.ac.ir/index.php/aacc/article/download/274/486