Mucopolysaccharidoses correspond to the set of congenital defects of metabolism in which there is a deficit for the processing of glycosaminoglycans, at the moment the risks of complications in the respiratory tract and neurological associated with abnormal deposits of intermediate metabolites of metabolism is high, for such Because of the high need for surgical interventions during childhood, this narrative review aims to explore the available evidence regarding the anesthetic management of patients with mucopolysaccharidoses, for which 2 cohort studies and 6 observational studies were selected, finding that the approach through multidisciplinary teams, premedication with anxiolytics, the use of videolaryngoscopy to protect the airway and the use of Propofol associated with dexmedetomidine in cases of patients with sanfilippo syndrome were recommended in the management of patients with mucopolysaccharides If, however, multiple residual biases were detected during the evaluation of the publications, which is why it is not possible to suggest such changes as evidence-based recommendations, new experimental investigations are required.