A girl weighing 2320 grams was delivered at 38 weeks to a mother with antenatal history of chickenpox in the fourteenth week of gestation. At birth, baby had cicatricial lesions with areas of induration and hypopigmentation on the torso, causing deformity of underlying rib cage (Fig. 1). The limbs were spared. She had bilateral chorioretinal atrophy with cataract in the left eye. Her sensorium was altered, and she had poor respiratory efforts requiring mechanical ventilation. Computed tomography of brain revealed bilateral thalamic calcifications and mild cerebral atrophy. Despite intensive care, baby died at fifty hours of life.

The risk of congenital varicella syndrome (CVS) after maternal varicella in the first 20 weeks of pregnancy is approximately 0.4 - 2%. Cicatricial lesions, neurological defects, ophthalmological manifestations and limb-shortening defects with muscular hypoplasia are the major clinical features in CVS. An important differential diagnosis for cicatricial lesions in a neonate is intrauterine Herpes Simplex Virus infection (distributed in dermatomal fashion but presence of active lesions, hypo- and hyperpigmentation, aplasia cutis, and/or an erythematous macular exanthema).