Malignant struma ovarii is a rare type of ovarian teratoma; only 16 cases with distant metastases have been reported previously. We report an extremely rare case of malignant struma ovarii metastatic to the cranial vault, which developed during pregnancy. A 28-year-old woman in the 26th week of pregnancy, who had undergone resection of an ovarian tumor 3 years previously, noticed a mass in her left frontal region that had enlarged gradually in 6 months. Magnetic resonance imaging revealed a massive extradural tumor growing through the cranium. Under fetal heart monitoring, the patient underwent total resection of the tumor, including the adjacent cranial bone and dura, and a healthy infant was delivered at full term. Pathological examination showed that the tumor was a follicular adenocarcinoma. Because there was no abnormality in the patient's thyroid gland, this tumor was considered to be a metastasis from the ovarian tumor, a malignant struma ovarii, resected 3 years previously. The management of brain tumor during pregnancy is also discussed.