Extrahepatic portal hypertension is commonly caused by portal vein thrombosis (PVT). Clinical manifestations of this may include splenomegaly, varices and ascites. Rare complications such as splenic artery aneurysms (SAAs) can present, especially in the presence of portal hypertension; however, such cases are poorly described in the literature and can therefore present a challenge in investigative work-up and management. The case of a young woman in her late 20s with chronic extrahepatic PVT from thrombophilic disease is presented in this report. She developed splenomegaly, thrombocytopaenia from hypersplenism, oesophageal varices and multiple large SAAs. She was successfully managed by splenic artery embolisation and creation of a portosystemic shunt followed by a splenectomy.