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Paragangliomas are neuroendocrine tumors that arise from sympathetic nerve ganglia. They can develop anywhere from the neck to the pelvis, but are most commonly found in the abdomen, particularly at the aortic bifurcation or in the periaortic region. Malignant paragangliomas account for 29–40% of cases. We report a case of 36-year hypertensive female presented with and right flank pain and accelerated hypertension. On evaluation she was diagnosed to have non unctioning kidney due to malignant pelvic paraganglioma with right ureteric encasement. We believe our case is one of the first reported in literature as rare presentation of malignant paraganglioma presenting as nonfunctioning kidney and accelerated hypertension.