SUMMARYA male infant with the features of Adams‐Oliver syndrome is described. These features included severe aplasia cutis congenita (ACC) of the scalp involving both skin and cranium, a shortened right foot with talipes equinovarus, extensive cutis marmorata telangiectatica congenita and also haemangiomas and ulceration of the abdominal skin. Exposure of the dural membrane was associated with infection, hyponatraemia and finally localised necrosis with herniation of brain and fatal cerebral haemorrhag