Idiopathic non-cirrhotic portal hypertension can emerge due to a varied spectrum of underlying and contributory factors, presenting in the form of abdominal distention as the initial symptom encountered. Often, a patient remains asymptomatic to the underlying cause and seeks medical care for their abdominal enlargement. As the portal hypertension continues to progress, ascites begins to develop due to a history of portal vein thrombosis being sufficient to increase splanchnic blood flow in a portal hypertensive pattern. We present a rare case of ascites in a non-cirrhotic patient due to portal vein thrombus with underlying myeloproliferative disease of primary myelofibrosis.