Background Paragangliomas are rare neuroendocrine tumors, especially in the bladder. Although many paragangliomas are non-functional, functioning paragangliomas present with many non-specific signs and symptoms, such as hypertension and headache, complicating their diagnosis. Here, we report a case of micturition-induced severe headache associated with a bladder paraganglioma. Case description: This report describes a severe headache disorder triggered by micturition and associated with a bladder paraganglioma in a middle-aged woman. Her pain occurred consistently after micturition, beginning from the left side of the nose and gradually extended to the forehead and the parietal and occipital regions. The headaches lasted 5–10 min. Removal of the paraganglioma completely eliminated the patient’s pain syndrome. Conclusion This case elucidates the association between micturition-triggered headaches and bladder paragangliomas. The presence of a post-micturition severe headache should suggest the possibility of a bladder paraganglioma.