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Pheochromocytomas and paragangliomas are neuroendocrine tumors that arise from chromaffin cells of adrenal medulla and extra-adrenal paraganglia, respectively. The recurrence of these neuroendocrine tumors as a jejunal mass causing obstruction in the small intestine is an exceptional entity. The present study reports the case of a 70-year-old male who presented to the Emergency Department of Bezmialem Vakif University Hospital with abdominal pain and vomiting. The patient possessed a history of left nephrectomy due to malignant pheochromocytoma that had invaded into the left kidney eight months prior to presentation. Bowel obstruction was diagnosed and the patient underwent a laparoscopic procedure. Partial resection of the jejunum was performed and immunohistochemical studies revealed the lesion to be malignant paraganglioma. The majority of paragangliomas are chemo- and radioresistant. Surgical excision remains the primary treatment. Metachronous paraganglioma arising from the small intestine is an extremely rare entity and may be a relevant consideration in patients presenting with bowel obstruction.