Pheochromocytomas and paragangliomas are a rare cause of sudden death. We present the case of a 34-year-old male who died suddenly. He had been diagnosed as having diabetes mellitus five days previously and was hypertensive. At autopsy he had a 3.5 cm extra-adrenal mass. Histology of the tumor showed it to be a paraganglioma with the characteristic histological appearance. Postmortem biochemistry revealed a vitreous glucose of 5.9 mmol/L (106 mg/dL) with negative ketones. Urinary catecholamines were measured and revealed raised metanephrine:creatinine and normetanephrine:creatinine ratios. There may be genetic implications in patients with pheochromocytomas and paragangliomas as they are associated with a number of hereditary syndromes.