Growing teratoma syndrome (GTS) is a rare condition among patients with non-seminomatous germ cell tumors who present with enlarging metastatic masses during appropriate systemic chemotherapy in the context of normalized serum markers. This is an infrequent event in the progression of testicular tumors, and is even less common in the case of ovarian germ cell tumors. The pathogenesis of GTS is not completely understood and diagnosis can only be made with certainty after complete pathologic examination. Although histologically benign, GTS may present an enveloping growth with aggressive local expansion, which can be related to substantial morbidity and mortality. Surgery is the only recommended treatment and early recognition of this syndrome is essential as it offers hope for curative resection and avoids the use of ineffective chemotherapy. The authors present a brief review of the literature, along with the case report of a 37-year-old woman presenting GTS with liver involvement who was successfully treated by debulking surgery followed by major liver resection. This report demonstrates that complete surgical resection results in excellent disease control. More importantly, it highlights that clinicians need to be aware of the possible development of GTS when monitoring their patients with non-seminomatous germ cell tumors. These patients require coordinated care between oncologist, gynecologists, and general surgeons to obtain the best possible outcomes.