A case of Graves' disease occurring in a patient with hemiagenesis is presented. The detection of the rare occurrence of a congenital hemiagenesis is often made by either clinical symptoms of thyroid dysfunction or anatomical abnormalities such as nodular goiter. The symptoms of hyperthyroidism in the current case led to the diagnostic confirmation by scintiscanning and ultrasonography of an absent robe. Anti-thyroid antibody studies documented the presence of Graves' disease within the remaining lobe.