We present a case of a 24-year-old gentleman who presented with painless pan haematuria for 2 weeks. During the workup, he was diagnosed to have a retrocaval mass after a CT scan while cystoscopy revealed a polypoidal pulsating lesion in the left ureter. After surgical manipulation of the retrocaval mass, the blood pressure of the patient raised to 260/130 mm Hg. It was completely resected and diagnosed as extra-adrenalpheochromocytoma (paraganglioma) after histopathology. The lesion in the ureter was completely excised and fulgurated and diagnosed as anarteriovenous malformation. To the best of our knowledge, this is the first patient to be presented in the literature with this unusual combination.