Abstract: Surgery for giant gastrointestinal stromal tumor (GIST) is technically challenging and associated with a high risk of postoperative recurrence. This article reports the case of a patient with a 42 cm giant GIST who presented to the emergency surgery department of Peking Union Medical College Hospital with symptoms of abdominal distension, limited food intake, and bilateral lower extremity edema. Following admission, the patient developed progressive hemoglobin decline, suspected tumor rupture, and worsening bilateral lower extremity edema. Through close multidisciplinary team collaboration, radical resection of the tumor was ultimately achieved. This article also reviews previously reported cases of GIST larger than 30 cm, aiming to provide a reference for the management of giant GIST.