This case describes a 50-yr-old man who was admitted to the Urology Ward upon the suspicion of a left kidney tumor. As part of the pre-operative check-up, an ultrasound and computed tomography of the kidneys were conducted. The results confirmed the initial diagnosis. The postoperative diagnosis was extra-adrenal pararenal phaeochromocytoma (ePCC) with succinate dehydrogenase complex, subunit B (SDHB) gene mutation. During the follow-up, a second tumor was detected by 3,4-dihydroxy-6-F-18-fluoro-L-phenylalanine positron emission tomography/computed tomography F-DOPA-PET CT that resulted in another surgery with complete resection of the tumor. The patient and his family were counseled by a genetic laboratory and remain under surveillance.