Mucopolysaccharidoses are a group of inherited disorders of metabolism resulting in the deposition of mucopolysaccharide in various tissues. This leads to organ dysfunction and anatomical abnormalities which can be important to the anesthetist. These abnormalities result in airway difficulty and difficult intubation. We successfully performed endotracheal intubation in a case of mucopolysaccharidoses in a 9-year-old female patient using fiberoptic laryngoscopy.