Ectopic thyroid is a rare condition. Here we report an extremely rare case of parapharyngeal space ectopic thyroid, which has simultaneously found the papillary thyroid carcinoma of the eutopic thyroid.A 54-year-old woman was admitted to our hospital for a thyroid tumor and neck lymph nodes. CT and MR imaging revealed the presence of a thyroid right node, as well as a right parapharyngeal mass with a diameter of 2.5 × 2.3 cm. PET-CT was also performed to diagnose further, revealing that the suv metric of the PPS mass was 4.03. Considering that the mass was asymptomatic, we did not handle it at the first thyroid surgery. However, when the patient underwent a radioactive iodine scan before the radioactive iodine treatment, the imaging showed that the mass could intake the iodine. So, we arranged the second surgery for this mass, and the postoperative pathological examination confirmed the mass was well-differentiated thyroid tissue.Parapharyngeal ectopic thyroid with eutopic thyroid cancer is extremely rare. Preoperative imaging examination can significantly avoid the missed diagnosis of this disease. Surgical resection is recommended for the ectopic thyroid while the eutopic thyroid is found to be malignant.Copyright © 2023 Elsevier Ltd. All rights reserved.