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Agenesis of the isthmus of the thyroid gland with bilateral levator glandulae thyroideae is an exceedingly rare congenital anomaly involving the thyroid gland. This condition, characterized by the absence of the thyroid glands isthmus and the presence of bilateral levator glandulae thyroideae, is typically asymptomatic but may have clinical implications, especially during thyroid surgery. Diagnosis is achieved through imaging studies, and management is tailored to the patient’s thyroid health and specific needs, which may include medication, surgery, or a combination of therapies. This anomaly underscores the need for comprehensive pre-operative assessment in individuals with thyroid disorders and highlights the importance of understanding anatomical variations to ensure safe and effective healthcare