Mucopolysaccharidosis type I is an inherited metabolic disorder secondary to enzyme deficiency and an inability to break down glycosaminoglycans. The subsequent accumulation of a toxic intracellular substrate in the brain results in multiple findings, almost all of them are demonstrated in our case. These findings include high T2 signal in the white matter, dilatation of the perivascular spaces (mostly involving the basal ganglia), a dilated ventricular system, prominent extra-axial CSF spaces (mostly at the level of the temporal lobes), an empty sella, and mega cisterna magna.