Lipoma of the uterus is a rare condition usually developing in postmenopausal women. Clinical symptoms and physical signs are similar to those found in leiomyomas. The histiogenesis of these lesions is still unclear. We report a case of a pure intramural lipoma of the uterus illustrating characteristic morphological and histological findings. Additional immunohistochemical studies underline the lipomatous but not leiomyomatous nature of the tumor.