A 33-year-old woman of giant broad ligament leiomyoma with myxoid degeneration was misdiagnosed as ovarian tumor. The patient underwent a transabdominal myomectomy and developed a pelvic infection after operation diagnosed with contrast-enhanced ultrasound. She was cured and ultimately discharged after symptomatic treatment. Only 21 cases of giant broad ligament leiomyomas with a diameter larger than 12 cm were included. The present systematic review aimed to increase awareness of the clinical characteristics and treatment methods of giant broad ligament leiomyoma, and reduce the rates of misdiagnosis and postoperative complications.