Struma ovarii (SO) is a rare form of ovarian tumor, which is defined by the presence of thyroid tissue comprising more than 50% of the overall tumor volume. The vast majority of the variants of SO are benign; however, malignant tumors have been reported in a small percentage of cases. An aggressive multimodality approach using ovarian cancer staging laparotomy, total thyroidectomy along with radioactive iodine-131 ablation, and thyroxin suppression therapy has been shown to safely treat malignant SO both its initial presentation as well as in the event of any subsequent recurrence with excellent efficacy and possibly better oncological outcomes. The rarity of the disease and the lack of evidence surrounding its management and prognosis continue to remain a challenge to the treating clinician. We present a unique case of malignant SO with an incidental synchronous association of follicular variant of papillary carcinoma of the cervical thyroid gland, this is possibly the second case reported in the English language literature.