Gastrointestinal stromal tumor (GIST) is the most common soft tissue sarcoma of the gastrointestinal tract. Surgery is the primary treatment of choice for patients with localized or potentially resectable GIST, whereas imatinib is the main treatment for patients with advanced, unresectable, or metastatic GIST. Neoadjuvant treatment with imatinib is not routinely recommended unless there is significant justification for reducing the tumor size in order to improve surgical outcome. The authors describe a case of a 48-year-old man who confirmed huge rectal GIST, and received successful neoadjuvant treatment with imatinib for preserving anal sphincter function followed by transanal excision.