Fucosidosis is a rare, autosomal recessive lysosomal storage disorder caused by a severe deficiency of a-L-fucosidase. Patients usually have some problems with glycoprotein storage in the brain and other organs, and some structural abnormalities that need special consideration in anesthesia. It has 2 types, the early onset or infantile, and the juvenile. Here we present an 8-year old girl with deformities in the maxillofacial region, with big tongue, small and retracted chin, saddle nose, and short neck that could not be extended, causing difficult intubation, and congenital cardiac problems requiring a special anesthetic strategy.