The clinical and laboratory findings in six Jamaican infants with the exomphalos-macroglossia-gigantism syndrome are described. The incidence was calculated as 1:13,700 births. There was no family history of similar disease in any case and no evidence of chromosomal abnormality, virus infection, or infantile hypoglycemia. Three infants died, and two showed typical histological features in the kidneys and adrenal glands.