The Authors report a case of a voluminous abdominal paraganglioma and a review of the literature on the surgical management of paragangliomas. CASE REPORT: A 58 years old woman was admitted to our Institution with acute endocrine impairment and simultaneous intestinal necrosis resulting from the mass effect of the tumor on the mesenteric vessels. Clinical and biochemical preoperative work up posed the suspect of paraganglioma. Previous intensive care treatment, the patient, underwent surgical resection of the tumor and left hemicolectomy, with immediate remission of both endocrine and abdominal syndromes. Histological examination confirm the preoperative clinical hypothesis. Paragangliomas are rare tumors of the extra-adrenal chromaffin tissue. A paraganglioma may be discovered in the absence of any symptom. When symptomatic, the clinical findings are related to the hypersecretion of catecholamines or to the compression of several anatomical structures from a growing mass. Combined acute syndromes of hormonal impairment and contemporaneous mass effect, as in our case, are exceptional. The diagnosis of the secreting forms may be obtained on the basis of biochemical dosage of serum and urinary catecholamines and metanephrines. The assessment of malignancy for paragangliomas is not always feasible. Several imaging, cytological and histological parameters have been proposed as predictors of malignancy, but the only element widely accepted is the existence of distant metastasis. Surgery represents the treatment of choice for paragangliomas. Benign forms, catecholamine secreting forms and tumors compressing neighbouring anatomical structures can be treated surgically with success. In general, an accurate follow-up after complete resection must be considered.