Introduction: Thromboembolic complications represent an important cause of mortality in patients with nephrotic syndrome, with renal vein thrombosis being the first historically described. Case description: A 55 year old male patient was referred to our Cardiology Clinic from a Nephrology department, where he was being investigated for newly diagnosed nephrotic syndrome, accusing intense shortness of breath and fatigue which appeared 6 days earlier. He recounted a similar episode before the initial onset of symptoms, which had been considered an interstitial pneumonia and treated accordingly with antibiotics. After admission, his ECG tracing, blood tests including D-dimers, troponin I and NT pro-BNP, and thoracic CT scan permitted the diagnosis of acute bilateral central and distal pulmonary embolism, for which he received curative intravenous anticoagulant therapy. Initial echocardiographic evaluation revealed acute right ventricle dysfunction, right ventricle hypertrophy and pulmonary hypertension. Further imagistic tests unveiled inferior vena cava thrombosis extending to the left renal vein, with no signs of neoplasia or deep vein thrombosis. Antithrombin III levels were normal, with slightly decreased levels of protein S, possibly in the context of acenocoumarol therapy. At the 2-months follow-up, the patient presented with persistent dyspnea (NYHA class III) and improved exercise capacity, while showing persistence of pulmonary hypertension and normalized right ventricular function. Results: Given the clinical and imagistic aspects of the case (thromboembolic pulmonary hypertension in a symptomatic patient – functional class III, proximal distribution of the emboli in the pulmonary arteries and lack of major comorbidities, with the exception of chronic renal disease stage IIIA, which is also a curable case of the pulmonary embolism), we consider that the patient may benefi t from pulmonary endarterectomy, for which he is referred to a specialized center. Conclusions: Thromboembolic pulmonary hypertension is a treatable form of the disease, which emphasizes the importance of early diagnosis and referral. Nephrotic syndrome and its procoagulant status as a risk factor for recurrent pulmonary embolism and possibly for thromboembolic pulmonary hypertension should be detected and treated and does not represent a contraindication for pulmonary endarterectomy.