A case of chromaffin functional paraganglioma of the organs of Zuckerkandl is reported in a 17-year-old girl, with severe hypertensive retinopathy (K-W grade Ⅳ) and fluctuating hypertension. Laboratory findings revealed increased VMA in urine and positive regitine test with diabetic oral glucose tolerance curve as well. Simple removal of a mass in an area of organs of Zuckerkandl resulted in complete alleviation of clinical symptoms for 2-year -follow-up period.