A unique partial anomalous pulmonary venous return in combination with other rare malformations such as annular pancreas and a persistent umbilical vein was discovered in a female Caucasian cadaver during an anatomical dissection at the Paracelsus Medical University in Nuremberg, Germany. The pulmonary anomaly comprised of the aberrant left superior pulmonary vein connecting the superior lobe of the left lung with the left brachiocephalic vein resulting in a left to right shunt. An annular pancreas without any signs of probable symptom causing duodenal compression was additionally found. To complete the constellation of malformations, a persistent umbilical vein connecting to the inferior branch of the extrahepatic left portal vein running in the round ligament fissure of the liver was also observed. This rare constellation of malformations has been illustrated and thoroughly discussed with the currently available literature to develop a hypothesis for the genetic and developmental background.