Lujan-Fryns syndrome (X-linked mental retardation with marfanoid habitus syndrome) is characterised by a marfanoid habitus becoming evident after puberty, typical craniofacial dysmorphism and behavioural problems. Recently, mutations in the MED12 gene and in the UPF3B gene have already been described as the cause of Lujan-Fryns syndrome. Lujan-Fryns syndrome was first reported by Lujan in 1984 and Fryns offered other features of this syndrome in 1987. Craniofacial features in Lujan-Fryns syndrome include prominent forehead, long narrow face, small mandible, maxillary hypoplasia, long nose with high and narrow nasal bridge, short and deep philtra, thin upper lip, highly arched palate, receding chin, and low-set retroverted normal shaped ears. Complete or partial agenesis of corpus callosum, ascending aorta aneurysm and ventricular septal defects are other manifestations of Lujan-Fryns syndrome. The prevalence in the general population is not known but affects predominantly males. There is no available treatment for Lujan-Fryns syndrome, patients need special education and psychological support. Diaphgram hernia and pulmonary hypoplasia are the most common anomalies in this syndrome. Because of these anomalies. limited pulmonary reserves and marked facial dysmorphism make it difficult to have a secure airway. Furthermore, the presence of cardiovascular malformations can lead to significant problems during anaesthesia practice.