Congenital Graves disease has been described as a transient disorder in which the mother has or has had hyperthyroidism. Experience with four affected children to ages 5 to 9 years and a review of published cases led us to conclude that long-acting thyroid stimulator (half-life, six days) is not the cause of the disease. This disease occurs in infants from families with a high incidence of Graves disease, and, in many, hyperthyroidism persists for months or years. The pathogenesis of Graves disease is unknown, and the simplistic maternal-to-fetal humoral theory is not a suitable explanation for congenital Graves disease. If Graves disease is considered in the larger perspective than the maternal-fetal unit, a pattern of inheritance is apparent, ie, an autosomal-dominant trait with a predilection for the female individual.