Rare diseases and syndromes are of special interest to pediatric anesthesiologits, as each of them has very specific anesthesia concerns. Knowledge of the pathophysiology, symptomatology, treatment options of these diseases and tailored anesthesia management forms the basis of provision of safe anesthesia care to these groups of children. Mucopolysaccharidosis are a group of lysosomal storage disorders. They are caused by the total or partial deficiency of one of the eleven enzymes involved in the metabolism of glycosaminoglycans. This deficiency leads to gradual accumulation of glycosaminoglycans in the lysosomes leading to permanent, progressive cellular damage which affects appearance, physical abilities, organ and system functioning and mental development. From the anesthesiologist point of view, these patients have problems with airway management and positioning. Few anesthesiologists get to routinely care for these patients. But individual patients undergo multiple surgical procedures for improvement in quality of life. We present a case of an 11-year-old girl with MPS posted for herniotomy.