Fetal goiter,a rare congenital malformation,is an enlargement of thyroid gland,which may have symptoms of increased thyroid function,decreased thyroid function or normal function.Thyroid function abnormalities can result in neurodevelopmental impairments.Besides,a large goiter may present with compression symptoms,such as dysphagia caused by the compression of esophagus and dyspnea caused by compression of airway,which lead to adverse pregnancy outcomes as premature delivery,dystocia,and fetal death.Ultrasound can reveal the thyroid directly and assess fetal growth,is the most important examination during pregnancy.At present,administration of oral drugs in pregnant women is the mainstay treatment for fetal goiter in the country.However,owing to rarity of the disease,there is currently no unified standard and guidelines for its diagnosis and treatment.The article reviews developments in the diagnosis,treatment and monitoring of the fetus with goiter during pregnancy.