Ear Nose Throat (ENT) surgeons are very much accustomed to common diseases in ENT. But it is challenging when it presents as a syndrome. At that time, striking of the correct syndrome can guide to proper and prompt treatment. Syndromes involving other systems are dealt with separately. ENT surgeons need to know the ENT features of common syndromes as they are likely to meet patients with these conditions in their clinical practice. The spectacular advances in basic and clinical genetics during the past two decades have brought congenital malformations and inherited disorders to the forefront of medical attention and care. The complete evaluation and treatment of these disorders can be challenging as well as rewarding. Management of congenital or acquired hearing loss; adequate speech and language education for maintenance of communication; airway management; swallowing exercises, and reconstructive surgery of head and neck are the primary modalities of treatment by an otolaryngologist. Major collaboration occurs between the otolaryngologist and the audiologist, speech therapist, maxillofacial prosthodontist, oral surgeon, and pediatrician. Most of the syndromes show multisystem involvement. Multidisciplinary collaboration plays an important role in the treatment. Referral to geneticist should be considered for molecular genetic testing and genetic counselling. This book provides a brief list of the more common ENT-related genetic and non-genetic syndromes as well as eponymous signs.