Background: Growing teratoma syndrome (GTS) is rare and is defined as an enlarging mature teratoma that arises during or after chemotherapy for a malignant germ cell tumor, with normalization of previously elevated serum tumor markers. Case: A 30-year-old nulliparous Japanese woman was diagnosed as having a stage IIIa immature teratoma. After fertility-sparing surgery, she received 4 cycles of chemotherapy consisting of cisplatin, etoposide and bleomycin. Thereafter, she successfully gave birth twice. Eight years after the initial surgery, a mass mimicking an adrenal tumor was resected laparoscopically. Surgical specimens revealed a mature teratoma, and she was diagnosed as having GTS postoperatively. Conclusion: Clinicians should consider that GTS may present late, even after pregnancy. Therefore, long-term follow-up of patients treated for ovarian immature teratoma should be mandatory.