Intestinal pseudo-obstruction is a rare and relatively
unknown complication of phaeochromocytoma/
paraganglioma (PCC/PGL). Its pathophysiology can be
explained by the hypersecretion of catecholamines, which may reduce the peristaltic activity of the gastrointestinal tract. Clinically, this can result in chronic constipation, intestinal pseudo-obstruction or even intestinal perforation. We conducted a comprehensive literature search and retrieved 34 cases of pseudo-obstruction caused by either benign or malignant PCC/PGL. We also included a case from our centre that has not been described earlier. We conclude that intestinal pseudo-obstruction is a rare but potentially life-threatening complication of PCC/PGL. Intravenous administration of phentolamine is the most frequently described treatment when surgical resection of the PCC/PGL is not feasible.