Gastrointestinal stromal tumors (GIST) comprise a rare group of neoplasms of unpredictable malignant potential with an annual incidence of 4/million persons. The stomach is the most common site of occurrence in the gastrointestinal tract. A combination of prognostic factors (patient age, histologic grade, mitotic rate, tumor size, and DNA analysis) have been utilized to predict their biologic behavior. Lymphatic spread of gastrointestinal stromal tumors is uncommon therefore a formal lymph node dissection is not standard surgical management. Consequently, complete surgical resection of the primary tumor is the most definitive treatment. An increasing number of cases have been reported utilizing a combination of laparoscopic and endoscopic techniques to resect these tumors. The manuscript will characterize the biologic behavior of gastrointestinal stromal tumors of the stomach, discuss the preoperative evaluation and minimally invasive surgical management of these patients, and review recent, encouraging adjuvant treatment strategies.