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Among endocrine glands, thyroid gland is well known for its developmental anomalies ranging from common to rare. During routine dissection of the male cadaver, agenesis of isthmus of thyroid gland in the midline was noted. It resulted in two lateral lobes with two pyramidal lobe and levator glandulae thyroidae. There is no anastamosis of blood vessels between the right and left lobes as normally seen. The agenesis of isthmus of thyroid gland is extremely rare and very few cases have been reported in literature.