Fertility-sparing management of endometrial stromal sarcoma has been demonstrated, but reports of pregnancy after such management are rare in our current body of literature. A 16-year-old nulligravid adolescent girl presented with symptoms of menometrorrhagia and was found to have a 17-cm uterine mass. The patient underwent local resection of the mass with uterine reconstruction. Pathology revealed a low-grade endometrial stromal sarcoma. She was placed on high-dose daily megestrol acetate therapy and remained disease-free for 8 years before achieving pregnancy spontaneously. The patient underwent an uncomplicated pregnancy until 34 weeks of gestation, when she presented in preterm labor and underwent cesarean delivery of a liveborn male neonate, with no evidence of disease recurrence. Fertility-sparing management and close follow-up of low-grade endometrial stromal sarcoma may be a viable option for those desiring future fertility.