We report a typical case of congenital varicella syndrome following a maternal varicella infection during the 10th week of pregnancy. The presenting symptoms in the small gestational age baby were: cicatricial skin lesion, Horner's syndrome,dysphagia with resulting aspiration pneumonia and delayed developmental milestones. Serologic studies revealed persistence of varicella zoster virus IgG for at least 10 months. Although congenital varicella syndrome is rare, it should be kept in mind when counseling families where maternal varicella infection has occurred during the first 20 weeks of gestation. A maternal history of varicella infection, typical manifestations and serologic proof are crucial in the diagnosis of congenital varicella syndrome.