Malignant struma ovarii is a rare clinical entity that poses a therapeutic challenge, as there is no 'gold standard' of care. We aimed to develop evaluation and treatment guidelines by reviewing presentation and outcomes of the available literature. We present a 60-year-old female with papillary thyroid carcinoma arising in a mature teratoma, and our multidisciplinary approach to care and follow-up. We examined 59 cases for characteristics, including rates of metastasis and recurrence, and response to surgical and adjuvant treatment. We found higher rates of metastasis and recurrence than traditionally reported, and found no recurrence in patients treated with oophorectomy, thyroidectomy, and I-131 radioablation. A multimodal approach to the treatment of malignant struma ovarii may improve survival and decrease risk of recurrence.