Cotyledonoid dissecting leiomyoma ("Sternberg tumor") is an unusual type of a benign uterine smooth-muscle tumor with a distinctive gross appearance. A 57-year-old woman (gravida 7, para 6), in menopause for 3 years, presented with a 3-4-year history of increased pelvic pain. The transvaginal ultrasound scan showed a 2.5 cm sized heterogenic mass in the uterus cavity and 4 × 5 cm sized irregular contoured mass in the right lateral aspect of the uterus. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed with removal of the pelvic tumor extension. Definite pathologic examination result was cotyledonoid dissecting leiomyoma of the uterus with intravascular growth. The patient is receiving follow-up care every 6 months, and she has no evidence of disease after 22 months of follow-up. The worrying appearance of the gross specimen is often mistaken for malignant or non-uterine lesions that may result in overtreatment. It is important to be aware of this entity to prevent overly aggressive treatment for this benign smooth-muscle neoplasm.