Meninigo-encepahlocoele (MEC) is a common neurosurgical operation. The size of MEC may vary which has bearing with its management. The association of MEC with micrognathia and microcephaly is rarely reported. The association poses special problem for intubation and maintenance of anaesthesia. Giant MEC may lead to significant CSF loss resulting in hemodynamic alteration. The prior knowledge and care in handling the patient can avoid minor as well as major complications.