Mucopolysaccharidosis (MPS) refers to a group of disorders characterized by the inability to breakdown glycosaminoglycans (GAG) in lysosomes. There are seven types—MPS I, II, III, IV, VI, IX—with several subtypes, each with their own spectrum of enzyme defects and severity of disease. MPS is a progressive disease, as GAG continues to build up over time affecting almost all systems in the body. Patients with MPS require multiple surgeries during their lifetime. However, this is a vulnerable population for anesthesia care with increased risk due to multiple factors. Primary difficulty lies in the airway management which may be very difficult manage, and as the patients grow older the difficulty increases (typically) In addition, patients with MPS are at a high risk for spinal cord injury both from the surgical procedure as well as the anesthetic itself. Treatments for these conditions are evolving as they are researched and developed. As of now the treatments slow or improve some aspects of the disease processes but not others. Anesthetic risk is substantial in this group of patients and an understanding of such risks and how to prevent or minimize them, is critical to safe care of these patients.