Carney complex is a rare autosomal-dominant familial tumor syndrome that involves the triad of myxoma, mucocutaneous pigmentation, and endocrine overactivity. To the best of the authors' knowledge, there are no reports of multiple fusiform aneurysms coinciding with atrial myxoma. The authors report the case of a 38-year-old woman with typical Carney complex who had multiple skin myxomas, endocrine abnormalities, and multiple brownish perioral lesions. Multiple fusiform aneurysms were also discovered after the recurrence of atrial myxoma. During a follow-up period of > 10 years, there have been no angiographic changes in the aneurysms and no progression of symptoms.