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Conventional transanal excision for a very low gastrointestinal stromal tumour. Gastrointestinal stromal tumour (GIST) represents a rare tumour entity, which has been more intensively investigated during the last decade. The rectum as the primary site of GIST is even uncommon. The space constraints in the pelvis renders optimal oncological surgery demanding and proximity of these lesions to the anal sphincter enhances the potential morbidity of any radical surgery. We herein report on a young patient with a >5 cm rectal GIST localised at 1 cm from the anorectal junction.