The case of a neonate with cutaneous lesions consistent with epidermal
naevi is presented. In addition to typical epidermal naevi, this baby
had an unusual, bullous form of aplasia cutis congenita. Although apl
asia cutis has been described as bullous and has been found in associa
tion with the epidermal naevus syndrome, both of these occurrences are
rare in medical publications. This case illustrates an unusual presen
tation of epidermal naevi with bullous aplasia cutis congenita and rai
ses difficult diagnostic and counselling issues.