Limb body wall complex (LBWC) is a rare polymalformative syndrome. The majority of cases presented in the literature have been diagnosed in the second or third trimester of pregnancy. The authors present a case of LBWC diagnosed in the first trimester of pregnancy. The combination of two- and three-dimensional ultrasounds proved to be useful for establishing the diagnosis and differentiating from other abdominal wall defects. The diagnosis was followed by therapeutic abortion at 14 weeks’ gestation, and the pathological report confirmed the diagnosis.