Mucopolysaccharidosis type III (MPS III), also known as Sanfilippo syndrome, is an autosomal recessive, neurodegenerative lysosomal storage disorder. Substantial challenges for airway management and endotracheal intubation are predictable due to the MPS patients' specific phenotypic, facial, and airway characteristics. In studies concerning various types of MPS, the incidence of difficult endotracheal intubation ranges between 28%-44%. This study intends to present a case with MPSIIIA, who aspirated her tooth during endotracheal intubation due to acute respiratory failure. This study reports a 12.5 year-old girl who presented with MPSIIIA and aspirated her tooth during endotracheal intubation due to acute respiratory failure resulting in lung lobe segmentectomy. The majority of MPS patients’ intubation and airway maintenance are hard and get even more difficult by age because of their mental retardation and oral health deteriorating, which may require tracheostomy.