Paragangliomas are extra-adrenal pheochromocytomas that arise from specialized neural crest cells. They are distributed anywhere from the upper neck to the pelvic floor, and they are classified on the basis of their anatomic origin. Functioning paragangliomas can cause the same clinical manifestations as pheochromocytoma, such as hypertension, diabetes mellitus, hyperadrenergic spells and so on. We experienced a retroperitoneal paraganglioma that was found in 66 year-old male who suffered from intractable constipation, and his constipation was caused by paralytic ileus and uncontrolled hyperglycemia. After he was diagnosed, removal of the paraganglioma was done and his clinical symptoms and sustained hyperglycemia were successfully resolved.