Gastrointestinal stromal tumours (GIST) are the common mesenchymal tumour of gastrointestinal tract which origin from interstitial cell of cajal (ICC) or its precursor cell. These tumours arise rarely from structure other than stomach like ileum, mesentery etc. This is a case report of 65-year-old female with malignant GIST arising in ileum part of intestine. On surgery a large 10 x 9 x 6 cm intraluminal bowel mass, with other small nodules resembling lymph node present on and adjacent to it. Histopathology study of mass confirmed to be the malignant GIST which show positivity for IHC like C-kit, Vimentin, SMA. We report this case due to very rare occurrence of GIST in ileum location.
