Congenital hypothyroidism (CH) is the leading cause of preventable mental retardation. It is mainly due to thyroid dysgenesis or dyshormonogenesis with normally located gland, and detected at birth in developed countries by systematic neonatal screening. The early treatment of patients with CH has successfully improved the prognosis and management of this disease. An increase in the incidence of congenital hypothyroidism with a normally located gland has been reported worldwide over the last three decades. The etiology of CH with a normally located gland remains elusive and about half of them demonstrate spontaneous resolution of CH within a few months (transient CH). They highlight the need to reevaluate thyroid function during follow-up.