Neonatal erythroderma results from various underlying conditions and presents at birth or by 1 month of life. Infections, inflammatory disorders, and congenital ichthyoses are the common causes. Atopic dermatitis and congenital psoriasis may result in erythroderma at this age. Rarely, immunodeficiency disorders and inborn errors of metabolism may present at birth with intense erythroderma and failure to thrive. Establishing the underlying cause for erythroderma is difficult in neonates. Infections like staphylococcal scalded skin syndrome and congenital cutaneous candidiasis are treatable, and early diagnosis and therapeutic intervention ensure survival of the child. The ichthyoses group of disorders attains a definite morphological pattern subsequently beyond the neonatal period, e.g., lamellar ichthyosis, congenital ichthyosiform erythroderma, etc. Some of the congenital ichthyosiform syndromes like Sjögren-Larsson syndrome and Chanarin-Dorfman syndrome have multisystem involvement and require thorough screening. Hereditary metabolic disorders like multiple carboxylase deficiency and neonatal biotinidase deficiency present with recurrent vomiting, lethargy, ketoacidosis, and periorificial eczematous eruption simulating acrodermatitis enteropathica. Management of neonates with erythroderma requires combined care by neonatologists and dermatologists. The monitoring of fluid and electrolyte balance, prevention of hypothermia, and early diagnosis and treatment of sepsis are the basis of management. Disease-specific management should be started when the underlying cause is determined.