Headaches associated with papilledema may be both life-threatening as well as vision-threatening. This review will review the following clinical features: (1) the character of headaches associated with increased intracranial pressure; (2) the visual symptoms associated with papilledema; (3) the funduscopic findings of true papilledema versus pseudo-papilledema; (4) the role of ancillary ophthalmological testing such as visual fields and spectral domain optical coherence tomography; (5) the neuro-radiological evaluation of patients with headaches and papilledema; (6) the treatment of vision-threatening papilledema.