A 59-year-old male with type 4 pulmonary hypertension was admitted with diagnosis of ischemic stroke after withdrawal of systemic anticoagulation. Increased hypoxemia demanded invasive mechanical ventilation. Investigation confirmed embolization to the left medial cerebral artery and excluded conventional predisposing conditions to stroke. Deep vein thrombosis of lower limbs was excluded. CT scan after right side contrast injection showed pulmonary embolism (PE) and a duplication of SVC with filling of the four heart chambers and pulmonary arteries (Figure 1A,B). Left side upper body contrast injection confirmed a PLSVC, contrasting exclusively the left heart chambers (Figure 1C,D).