Pheochromocytoma originates from primitive neuroectodermal cell rest in adrenals and other paraganglion structures. The term composite pheochromocytoma is used in reference to pheochromocytoma where there are other components resembling neuroblastoma, ganglioneuroblastoma, ganglioneuroma and spindle cell sarcomatous tumour coexist. Here a 61 year old man with pheochromocytoma of right adrenal gland along with tumour component of MPNST-malignant peripheral nerve sheath tumour co-exist (Composite

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... pheochromocytoma) is being presented. The patient presented with left flank pain of one month duration along with complaints of Insomnia, anxiety, palpitation and persistent constipation of short duration.