We describe a case of concordant body stalk anomaly in monozygotic twi
ns that manifested with umbilical cord agenesis, evisceration of abdom
inal contents, and multiple gastrointestinal, genitourinary, and skele
tal anomalies. Body stalk anomaly in twin gestation is extremely rare
and poses an embryological interest. We suggest that the hypothesis of
'incomplete twinning' might contribute to the pathogenesis of this sy
ndrome complex.