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Pheochromocytoma is one of rare tumors of neuroendocrine. Most of the tumors are sporadic with about 1/3 of them caused by genetic mutations. Papillary thyroid carcinoma (PTC) from thyroid follicular cells is the most common malignant tumors. However, pheochromocytoma with PTC is rarely reported. Here we present a case report of an 41-year-old male patient found to have an adrenal pheochromocytoma combined with PTC, and PTC was diagnosed first with fine needle aspiration. The patient admitted to hospital, and surgery would be prepared when abnormal blood pressure rose, and operation was suspended. Further CT scan and blood biochemical examination were performed, and concurrent adrenal pheochromocytoma was considered. Multidisciplinary team decided to perform adrenal surgery priority. When the recovery of patient appeared after first operation, radical thyroidectomy was performed. Whether the co-existence of pheochromocytoma and PTC is coincidence or caused by RET gene or unknown inherited genetic mutations is unclear, so accumulation of studies with more combined cases should be done and genetic testing data are needed.