Primary retroperitoneal tumors are extremely rare tumors of this location. The authors present a case review of a 53-year-old patient operated for unexplained retroperitoneal neoplasm. No complications were recorded during the postoperative course and histology demonstrated extraadrenal paraganglioma structures, ranked among the above neoplasms. Preoperative hypertension subsided postoperatively, following the active neuroendocrinne tumor removal. The aim of this work is to present clinical and histopathological characteristics of this rare retroperitoenal neoplasm.

        Key words: extraadrenal paragangioma – primary retroperitoneal tumor – neuroendocrinne activity