A 4-year-old girl was seen in our department for erythroderma, palmoplantar hyperkeratosis, and scalp desquamation present since birth. The dermatosis had run an intermittent course, with exacerbations after infections and spontaneous remissions. A specimen from a skin biopsy performed at 1 year of age showed the characteristic features of psoriasis, findings that were confirmed in our biopsy specimen. Treatment with acitretin controlled the outbreaks. At 7 years of age she has developed, for the first time, plaque type psoriasis. Congenital erythroderma is an unusual form of psoriasis with a wide differential diagnosis.