Two children, one boy and one girl, each with solitary maxillary deciduous and permanent central incisors, normal height, and normal plasma growth hormone levels, are reported. Review of the literature reveals fourteen reports of patients with a similar dental anomaly. Of these, three are normal in height. The height and growth rate of patients with a single maxillary central incisor should be evaluated prior to growth hormone evaluation; such investigation should be undertaken only if the height is two standard deviations below the mean or if there is evidence of plateauing of linear growth.