Neoadjuvant therapy with Imatinib is the preferred treatment for rectal GISTs in order to reduce the aggressiveness of a surgical resection. At present, surgical resection does not require wide surgical margins. There is also a lot of controversy with regard to the need for a broad anatomical resection or if a local resection is sufficient and maintains maximum oncological safety and minimum morbidity and mortality. We present a case report of a rectal GIST with a good response to neoadjuvant treatment which allowed a transanal resection.