Parapagus (laterally fused), diprosopus (two faces), dibrachius (two upper extremities), dipus (two lower extremities) conjoined twinning is extremely rare. The coexistence of anencephaly with a contiguous spinal defect (craniorachischisis totalis) makes the present case one of the rarest of the published cases. In our case, it was difficult to make the final diagnosis by two-dimensional abdominal and vaginal ultrasound. Three-dimensional ultrasound was helpful for final diagnosis and post-abortal examination confirmed the prenatal ultrasound diagnosis. The heart, diaphragm, liver and perineum were all united. Fine dissection of the heart showed four vessels arising from the ventricles and a membranous type ventricular septal defect.