Two 4-month-old infants with signs of intracranial hypertension associated with vitamin A deficiency are reported. Intestinal malabsorption due to cystic fibrosis was the underlying cause for the hypovitaminosis A. The recognition of vitamin A deficiency was delayed because of failure to appreciate that bulging anterior fontanelle is a sign of deficiency of this vitamin. These cases serve to reemphasize this infrequently reported association and serve to remind the pediatrician that vitamin deficiency disease in the United States is of more than historical interest. Prompt recognition of this syndrome and appropriate therapy can prevent blindness and death.