The authors are reporting a typical case of congenital varicella syndrome following maternal varicella during the 17th week of pregnancy. At birth, the newborn showed necrotic bullae on the skin that healed later with characteristic scars. Other typical anomalies, i.e. hypoplastic limbs with muscular atrophy and clubfoot, intrauterine atrophy, dysphagia and anisocoria were also found. In view of the risk of serious malformations following intrauterine varicella infection attempts should be made to prevent varicella zoster virus infection during pregnancy.