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Congenital absence of a lobe of the thyroid gland is discussed and the literature is reviewed. Sixty-six cases have been gathered from the literature in approximately the past 100 years, and another six have been documented. Although no particular explanation has been forthcoming for the development of this anomaly, left lobe aplasia and predominance of occurrence in women have been noted. A variety of other thyroidal abnormalities have coexisted with hemiagenesis.