Abstract: Fetal limb-body wall syndrome (LBWC) is one of the malformations of fetal anterior abdominal wall, also known as fetal body stalk anomaly (BSA), which is caused by the failure of anterior abdominal wall closure. LBWC is a complex malformation combination, which may have many kinds of deformities, such as abdominal fissure, chest fissure, limb deformity, scoliosis, facial craniocerebral deformity, short umbilical cord and so on. Incidence of LBWC is low, the pathogenesis is unknown, the prognosis is very poor, and the fatality rate is very high. Once diagnosed, the pregnancy should be terminated as soon as possible, which generally will not affect the second pregnancy. Prenatal ultrasound plays an irreplaceable role in the early diagnosis of fetal LBWC, and it is the first choice for non-invasive examination at present. There is usually no chromosome abnormality in fetal LBWC, so routine chromosome examination is not recommended, but reasonable prenatal counseling and prenatal diagnosis are still necessary. This study reported a case of diagnosed fetal LBWC by prenatal ultrasound at gestational week of 13⁺² and reviewed the related literature.