Sjögren-Larsson syndrome (SLS) is a rare inborn error of metabolism characterized by congenital ichthyosis, intellectual disability, and spastic di- or tetraplegia. Ichthyosis, usually present at birth, may occur after the first year of life. It is an autosomal recessive condition due to defective fatty aldehyde dehydrogenase (FALDH) mapped to the SLS locus on chromosome 17.