In a male individual (HET25_501; Table 1) with heterotaxy comprising left sided inferior vena cava, joining right atrium, midline positioned liver, accessory spleen, and membranous duodenal stenosis, a compound heterozygous VUS in PKD1L1 coding for Polycystin 1-Like 1 was found. The gene discussed is PKD1L1; the disease is Duodenal stenosis.