Penetrating aortic ulceration (PAU) is part of the spectrum of acute aortic syndromes (AAS), and is defined as an ulcerated intimal disruption due to atherosclerotic disease. PAU may be simple, isolated and asymptomatic, or it may be symptomatic, aneurysmal and extensive; these may progress and lead to rupture. This review aims to evaluate the treatment options for PAU. Treatment options range from radiological surveillance, risk factor modification, best medical therapy and open or endovascular surgical repair. Patients with PAU are frequently older and comorbid with relatively damaged aortic tissue; this can make open surgical repair more challenging. Endovascular repairs for larger, symptomatic, aneurysmal PAU may be performed with acceptable outcomes. Complex endovascular repairs may be required depending on the pathology. Patients with small, asymptomatic, uncomplicated PAU may be safely followed up with surveillance. PAU with concomitant intramural haematoma (IMH) should be closely monitored to observe for resolution or progression following conservative and medical management strategies. PAU is a unique entity and its natural history should be studied independently to better understand appropriate management strategies. This research is currently lacking, and larger studies or registries may be helpful in optimising PAU management.