Congenital varicella syndrome (CVS) is a rare but deleterious consequence of primary varicella zoster virus (VZV) infection during pregnancy. Typical CVS stigmata are cerebral abnormalities, eye diseases and segmentally distributed, cicatricial skin lesions. In this paper the authors report on a male preterm infant, born at 30 weeks of gestation, who developed pustular skin lesions at the age of 4 weeks. The mother had suffered from chickenpox at 14 weeks of gestation. Apart from skin manifestations, critical bronchopulmonary dysplasia made the infant conspicuous. The VZV genome was detected in blood, respiratory secretions and skin lesions. At age 10 weeks he presented with extensive intestinal wall perforation, considered to be related to CVS, which finally led to death. This case shows for the first time the clinical course of CVS in a preterm infant. It illustrates the need for discussion of comprehensive VZV vaccination for seronegative women of childbearing age.