RÉSUMÉ
SummaryGastrointestinal stromal tumours (GISTs) are malignant mesenchymal tumours that originate from the interstitial pacemaker cells of Cajal. They are particularly rare, comprising only 5% of all GISTs, and often present at an advanced stage. Treatment for these tumours is still a topic of debate due to their low incidence and insidious location. A woman in her mid-70s presented with rectal bleeding and anal discomfort. A 4.5×4 cm anal GIST was diagnosed. A local excision was performed and the patient was further treated with tyrosine kinase inhibitors. At a 6-month follow-up, the MRI showed that she was disease free. Anorectal GISTs are unusual and tend to be aggressive. Surgical resection is the first-line treatment for primary, localised GISTs. However, the appropriate surgical technique for these tumours is still a topic of debate. Further studies are needed to fully understand the oncologic behaviour of these rare neoplasms.