Although atrial myxoma is the most common primary tumor of the heart, the synchronous occurrence of myxomas of the intestine and the heart has not been reported in the English literature. We report a case of a 47-year-old woman who presented with small bowel obstruction by a pedunculated mass that was found to be a myxoma after resection. A left atrial mass was found incidentally by a computed tomographic scan, and a diagnosis of atrial myxoma was confirmed after a second surgery. The cardiac myxoma showed classic histologic features, with tumor cells layered around vascular channels in an abundant myxoid matrix, while the small bowel lesion was less cellular. Immunohistochemical stains yielded identical results in both. No vascular involvement was noted at either site. This case supports the recommendation that a search for a cardiac lesion should be performed when a myxoma is identified at an unusual location.