Background: Congenital heart diseases rarely have a primary manifestation in adulthood. They are a rare cause of pulmonary hypertension in adults. Case presentation: A 70-year-old woman of Eurasian descent underwent emergency surgery for bowel ischemia. Her history of mild pulmonary hypertension likely correlates with a peculiar diagnosis of an anatomic anomaly on the postoperative x-ray and computed tomography scan. The central venous catheter was misplaced. Initial management consisted of removal of the catheter. The diagnosis, partial anomalous pulmonary venous return, may pose a clinical therapeutic dilemma. Conclusions: Partial anomalous pulmonary venous return is a potentially treatable cause of pulmonary hypertension. With the current trend toward more medical imaging, we expect this diagnosis to be made more often in the future.