Gastrointestinal stromal tumors are the most common mesenchymal neoplasms of the gastrointestinal tract but gastrointestinal stromal tumor arising from rectum is a rarity. Biopsy of the lesion and immunohistochemistry for CD117 confirms the diagnosis. Surgery remains the standard of care and only potentially curative therapy for patients with primary, resectable, localized gastrointestinal stromal tumor. However, chemotherapy with Imatinib is added in neoadju-vant or adjuvant form according to

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... linical situation. Case report: We present a case of rectal gastrointestinal stro-mal tumor in a 55 years old male who presented with bleeding per rectum and altered bowel habits of 2 years duration. Patient was managed with abdomino-perineal excision and total meso-rectal excision and permanent colostomy. Histo-pathological examination revealed gastrointestinal stromal tumor of rectum (spindle cell type) measuring 7.2 x 6.4 x 5.1 cm having mitotic index >10/50 HPF. Immunohistochemistry showed marked positivity for CD117. Patient was given ad-juvant chemotherapy with Imatinib and he is still disease free after 1 year of follow up. Conclusion: Although rectal gastrointestinal stromal tumor is extremely uncommon, it should be included in differential diagnosis of rectal mass because this tumour has different biology which guides its treatment. Although complete surgical resection with negative margins is the principal curative option for primary and non-metastatic tumors, more studies are needed for determination of treatment strategy of the rectal gastrointestinal stromal tumors.