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We report a case of paraganglioma of the appendix in a 78-year-old male who presented with right upper quadrant pain, gallstones, and nausea. To the best of these author’s knowledge, this is the one of the first cases of a pure paraganglioma of the appendix reported in the literature. The rarity of this disease makes it difficult to isolate clear predictors of malignancy. Lifelong follow up in patients with chromaffin cell tumors is mandatory. Immunohistochemical analysis is considered a crucial step in diagnosis of paraganglioma. These tumors stain positive for a wide variety of markers including synaptophysin, chromogranin, and S100, along with demonstrating a low nucleus to cytoplasm ratio. We do believe that an accurate diagnosis of paraganglioma of appendix was rendered.
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