Although first described almost half a century ago, parastomal varices are not easily recognised as a cause of stomal bleeding even though they occur in up to 5% of all people who have a stoma. The main challenges associated with this condition are diagnosis and management. For that reason, the aim of the present study was to perform a systematic review of all the available literature pertaining to this topic. The primary end point was recurrent variceal haemorrhage after a particular mode of management. Several secondary endpoints focused on means of diagnosis and pathological conditions of abdominal organs that could contribute to both the formation of these varices and the rate of re-bleeding. Sixty-six articles comprising 210 patients were analysed. Parastomal varices tend to be more frequent in men manifesting with bleeding in the fifth decade of life. The majority (72.0%) of patients who bleed from parastomal varices do so from an ileostomy. The most common pathology leading to stoma formation is ulcerative colitis (57.8%). Liver cirrhosis is the most common cause of portal hypertension leading to the development of parastomal varices and primary sclerosing cholangitis is in second place. A third of patients with parastomal varices also have co-existent oesophageal varices. There are no pathognomonic symptoms or signs of parastomal varices and only the minority of patients have a raspberry appearance of the stoma, visibly dilated submucosal veins and bluish discoloration and hyperkeratosis of the skin around it. Venous phase contrast angiography or portal venography is the most successful radiological investigation to confirm the diagnosis. The transjugular intrahepatic portosystemic shunt (TIPS) procedure has the highest success rate in preventing recurrent haemorrhage and local measures, either non-operative or surgical, are the least effective. Comparison of TIPS with non-operative and local surgical treatment groups produced a risk reduction in 4.60 and 3.85, respectively. Treatment of 1.37 people with a TIPS procedure prevents one person suffering from recurrent variceal bleeding and using TIPS can reduce the likelihood of re-bleeding by 78.5%. Surgical portosystemic shunting or embolisation alone leaves patients with approximately 50% chance of re-bleeding. Although TIPS has gained popularity over the last two decades almost three quarters of patients with parastomal varices are still treated with local measures as first-line management. Liver transplantation as a treatment of the primary cause of parastomal varices remains very rare.