The authors report 3 different cases of prenatal diagnosis of situs inversus associated with bowel malrotation. Heterotaxy existed in 2 cardiosplenic syndromes (1 left and 1 right isomerism), and 1 isolated situs inversus. Bowel malrotation was detected at birth by ultrasonography and intestinal contrast study. Patients underwent laparoscopic LADD's procedure and abdominal exploration in the neonatal period. The authors advocate neonatal screening and early surgical management of bowel malrotation in prenatally diagnosed heterotaxic syndromes.