In this report, we describe a 36-year-old man with the synchronous occurrence of multiple gastric gastrointestinal stromal tumors and multiple intestinal lipomas. Multiple, small, and well-circumscribed gastric gastrointestinal stromal tumors (3 mm to 2 cm) were present within the gastrectomy specimen. The gastrointestinal stromal tumors were composed of epithelioid cells that were strongly positive for CD117 but negative for S100 protein or smooth muscle-specific actin. Also, 17 small submucosal lipomas were identified in the duodenal portion of the gastrectomy specimen. Endoscopic follow-up of the patient revealed more than 20 additional lipomas scattered throughout the intestine. Several large intestinal lipomas were resected endoscopically and histologically confirmed. Although both multicentric gastrointestinal stromal tumor and gastrointestinal lipomatosis have been reported in association with a few genetic syndromes, there has been no report, to our knowledge, of their coexistence. We feel that the coexistence of multiple lesions of these 2 rare diseases in this relatively young patient may represent a novel syndrome.