Netherton's syndrome, a rare congenital disease of childhood, is characterized by variable cutaneous erythematous eruptions with different manifestations. A five-year-old boy, who presented with ichthyosis linearis circumflexa, atopic manifestations and pili torti, had spastic hemiparesia due to cerebral infarction. Netherton's syndrome can easily be misdiagnosed as Leiner's disease, generalized psoriasis or nonbullous congenital ichthyosiform erythroderma, especially in the neonatal period, because of its nonspecific clinical and histological features. Pediatricians should consider this syndrome in the differential diagnosis of the generalized erythematous skin disorders of childhood associated with various abnormalities.