Mucopolysarccharidoses (MPSs) is a rare group of disorders with a very high percentage of otolaryngologic symptoms and signs. Otolaryngologists play an integral role in the multidisciplinary approach to the diagnosis and management of many children with MPSs disorders. Children with MPSs are born without typical symptoms and develop a variety of symptoms over time. Otolaryngological manifestations of MPSs disorders may be especially considered adenotonsillar hypertrophy, otological problems, and airways problems. Otolaryngologic problems and their treatments evaluated on MPSs patients in this article.