SummaryThe term neonatal or infantile erythroderma designates a generalized erythe‐ma covering more than 90% of the body surface area of neonates and young infants. Erythrodermas are not rare in this age group and have been associated with more than 40 diseases of different degrees of severity.Regardless of the underlying illness, impending acute symptoms of an impaired epidermal barrier function (hypothermia, dehydration, sepsis) must be recognized and treated adequately. Most often patients have to be hospitalized for incubator care and, if necessary, intravenous rehydration and antibiotic therapy.Later a thorough diagnostic workup should be performed in cooperation with specialized centers in order to guarantee a precise diagnosis and the most effective treatment. In particular, potentially severe extracutaneous symptoms require rapid intervention and represent the main focus of this review.