BACKGROUND: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is a rare disorder associated with plasma cell disorder. Optic disc swelling can be present, and macular oedema has been reported once before. PATIENTS AND METHODS: Three patients with POEMS syndrome were investigated. Lumbar puncture and neuro-ophthalmic examination were performed on all patients. RESULTS: Visual symptoms included decreased vision, photopsia and blurred vision. Optic disc swelling was present in all three patients. Visual acuity was decreased in only one patient (0.2 in right eye, 0.7 in left eye) due to macular oedema, and another patient with normal visual acuity showed also a mild macular oedema on fluorescein angiography. In these two patients, elevated intracranial pressure was found (30 and 39 cm H(2)O, respectively). The macular oedema disappeared under steroids and acetazolamide therapy with visual improvement, but optic disc swelling remained. CONCLUSION: Optic disc swelling is frequent in POEMS and macular oedema may be more frequently present than previously thought. Interestingly macular oedema was detected in the two patients with increased intracranial pressure.