Uterine arteriovenous malformation (AVM) is a rare disease. However, it is important to make a rapid and precise diagnosis when it does occur, because life-threatening massive genital bleeding may occasionally be a symptom of this disease. In this case report, we present a case of uterine AVM diagnosed by means of ultrasonographic, magnetic resonance imaging (MRI) and angiographic studies. A 47-year-old Japanese woman was admitted complaining of severe hypermenorrhea that had been occurring for 5 years. Her last pregnancy was an uncomplicated term delivery at 32 years of age. Transvaginal and transabdominal ultrasonography demonstrated a thickening of the anterior wall of the uterine corpus with numerous cystic lesions. Color Doppler ultrasound, dynamic MRI and magnetic resonance angiography as well as pelvic angiography were useful for detecting the hypervascular lesions in this case. She was treated by a total hysterectomy and bilateral salpingo-oophorectomy under the diagnosis of uterine AVM. These noninvasive techniques should be performed initially when this rare disease is suspected.