Thyroid dysgenesis, the most common cause of congenital hypothyroidism, includes hypoplasia, ectopia, and agenesis. Agenesis may present as hemiagenesis, bilobar agenesis, or isthmic agenesis. A small ectopic thyroid tissue may sometimes be detected on the embryonal migration pathway of suspected bilobar agenesis cases. Comorbidity of hyperparathyroidism and thyroid dysgenesis has been a topic of interest. The case presented here is a pioneer in medical literature with a comorbidity of parathyroidism and ectopic thyroid tissue.