'Double cortex' is a rare hereditary dysontogenetic condition whose transmission is still undetermined. The great majority of cases are females; only one male has been reported in literature. We report the case of a 32 year old man with a severe mental retardation, short stature, dysonichia, disodontiasis, condropathy, skin abnormalities, endocrinopathy, double cortex, epileptiform abnormalities on EEG without clinical seizures and an abnormal karyotype.
