Objectives: To present the indications for myomectomy during pregnancy and to discuss complications possibly related and unrelated to the procedure. Method and Results: A 33-year-old patient at 18 weeks of gestation underwent removal of a 1,570-gram symptomatic fundic myoma. Histologically the patient had a leiomyomatous neoplasm of uncertain malignant potential. The pregnancy was continued under sequential observation with magnetic resonance imaging and ultrasound. At 36 weeks of gestation a healthy girl with an upper extremity limb defect was born via cesarean section. Follow-up of the mother and the child was uneventful. Conclusions: Certain known risk factors in pregnant women with myomas can predispose to complications during pregnancy. Women with such risk factors or women who have failed medical therapy should be offered the option of undergoing myomectomy as a pregnancy-preserving procedure.