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BackgroundUterine leiomyomas are the most common gynecological tumors in women of child-bearing age and premenopausal women, while benign metastasizing leiomyomas of the heart are rare.Case presentationWe report a rare case of metastasizing leiomyoma in the heart of a 54-year-old woman 10 years after a uterine leiomyoma was discovered during hysterectomy. Echocardiography, cardiac plain scan and enhanced MRI at presentation showed a soft tissue signal mass in the right ventricle. A large cardiac mass attached to the chordae of the tricuspid valve and later shown to be histopathologically consistent with uterine leiomyoma was successfully resected through a right atriotomy.ConclusionsOur case report highlights a rare type of tumor of the heart and suggests that metastasizing leiomyoma should be considered in the differential diagnosis of right-sided cardiac tumors. The complete surgical resection of the tumor was considered to be the best treatment.