A case of congenital short bowel syndrome with left acheiria, hemivertebra and dextrocardia is described. Dilatation of the fetal bowel was observed at the 24th week of gestation during a routine ultrasonic scan of a healthy 23-year-old primigravida from a non-consanguineous marriage. Amniocentesis and chorionic villus sampling were denied. The baby was delivered at 38 weeks of gestational age. After delivery, multiple anomalies were noted: left acheiria, congenital short bowel syndrome (15 cm in length of the ileum), pseudo-obstruction of intestine, dextrocardia, and hemivertebrae. We suspected these abnormalities might be due to a vascular accident or failure of lateralization during the early gestational period. To our knowledge, these combinations have not been reported previously in English literature.