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Hemiagenesis of the thyroid gland (THA) represents a rare congenital anomaly. It is characterized by the absence of one thyroid lobe, and sometimes the isthmus as well. It can occur with all kinds of other thyroid pathologies that may be present in the remaining thyroid lobe. A case of a 21-year-old male patient is presented; he sought a thyroid consultation because of hair loss, fatigue, and problems concentrating, thus raising the suspicion of hypothyroidism. Thyroid function was normal, but sonography of the thyroid gland revealed THA of the left lobe and the isthmus. The current knowledge concerning the genesis and the clinical consequences of THA are discussed based on the current literature.