The authors describe a case of paraganglioma of the sellar region in a young female patient with loss of vision and headache. She presented with amaurosis, depression, anxiety and amenorrhea. Clinical and radiological impression was that it was a meningioma or pituitary adenoma. She received bromocriptine with no reduction of the lesion. She developed panhypopituitarism, but with normal levels of prolactin. It was resected and histological examination revealed nests of large cells with moderate nuclear pleomorphism, vesicular nuclei with occasional nucleoli. There were rare mitotic figures, but no necrosis. Immunohistochemistry was positive for synaptophysin, chromogranin A, and neuron-specific enolase with a few sustentacular cells positive for S100. The Ki67 proliferation was 1-2%. All pituitary hormonal antibodies were negative as well as GFAP, AE1/AE3, p53 and EMA. Paragangliomas affecting the sellar region are extremely rare and might be due to the presence of remnants of paraganglionic tissue or abnormal migration. The patient's post-operative diabetes insipidus remains under medical control.

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