During a period of 9 years, 71,500 patients underwent thyroid investigations at our department. Sixteen patients with thyroid hemiagenesis, 13 women and 3 men, were seen during this period. Fifteen had no left lobe and only 1 had no right lobe, the isthmus was present in 5 patients. Associated thyroid diseases of the lobe that was present could be observed in 11 patients (9 diffuse or nodular goiters, 2 thyroid autoimmune diseases). One patient was hyperthyroid and 7 were hypothyroid. Hypothyroidism associated with hemiagenesis has rarely been reported in the literature. In our survey, the high percentage of hypothyroidism may be explained by coexisting iodine deficiency, which could be verified in 4 hypothyroid patients. Ultrasonography is the key investigation to diagnose thyroid hemiagenesis. Fine-needle aspiration biopsies, laboratory tests, and scintigraphies are useful to diagnose other diseases within the remaining lobe or to visualize ectopic thyroid tissue. Review of the literature, including our cases, presented a total of 256 patients with thyroid hemiagenesis. Its prevalence can be estimated between 1:1900 and 1:2675. Left to right ratio of thyroid hemiagenesis is 3.6:1 with an isthmus present in 44%. The female-to-male ratio is 3:1; however, the larger number of females is probably based on a bias due to a female predominance of the populations investigated. On the basis of an equal distribution of both sexes, the female-to-male ratio of thyroid hemiagenesis would be only 1.3:1 in our survey.