Conjoined twins have an estimated incidence of one in 50,000 to one in 100,000 births. The first cases of prenatal diagnosis of conjoined twins using B-mode ultrasound were reported in the 1970s, followed by prenatal real-time ultrasonographic diagnosis shortly thereafter. Since then, real-time ultrasound has been the most reliable method in establishing prenatal diagnosis of conjoined twins and in detecting associated anomalies. Moreover, recent improvements in real-time ultrasound technology -in some cases coupled with use of Magnetic Resonance Imaging (MRI) -have made early diagnosis of conjoined fetuses in the first trimester possible. Prenatal diagnosis of conjoined fetuses is considered essential for further management. Parents need detailed counseling in order to decide among various management options, which include the following: (a) pregnancy continuation and scheduled neonatal surgery, (b) termination of pregnancy, and (c) multifetal pregnancy reduction or selective fetocide in cases of high-order multifetal pregnancies with a component of conjoined fetuses. An interdisciplinary medical team, involving obstetricians, neonatologists, pediatric surgeons and anesthetists, should carefully approach and assist parents in their decisions. In special cases, additional consultation may be given by other specialists, even from specialized centers in other countries, depending on the area of conjunction, the involvement of other organ systems, and the presence of associated anomalies.