Objective We describe clinical presentation, therapy and outcome of 47 patients with demyelinating disorder seen at our Institute from 2001 to 2014. Methods Patients were studied with clinical and MRI follow-up, emathological investigations for infectious agents, CSF analysis and electrophysiologic data. Neuroimmaging of all patients were reviewed considering the new 2013 IPMSG criteria. Results 24 females and 23 males (age 6 months to 17 years at onset): 25/47 were classified as ADEM, 9/47 as NO, 8/47 as CIS and 5/47 as MS. Symptom at onset were: 20/47 fever, 31/47 visual impairment and/or dyplopia, 25/47 encephalopathy, 17/47 motor symptoms, 12/47 ataxia, 12/47 dizziness, 11/47 headache,10/47 vomit, 9/47 meningeal signs, 9/47 sensitivity symptoms, 7/47 sphynteric dysfunctions and 3/47 seizures. Only nine of these patients had a monofocal presentation. Fortysix patients were treated firstly with pulsed high dosage steroid therapy, followed by tapering with oral steroids. Eight patients of 45, who performed a first line therapy with steroids, did not respond and were subsequently treated with IVIG, with good response in 7/8, one responded to further plasmapheresis. Only three of our patients needed admission in Intensive Care Unit with ventilatory support. After at least 6 months of follow-up (range: 6 month-7 years), all patients were clinically and neuroradiologically revalued and 8 were classified as Optic Neuritis, 24 as ADEM, 5 as CIS and 10 as MS. Conclusion Most of our patients with pediatric onset of demyelinating disease presented with an ADEM like attack. The most frequent symptom at onset was visual impairment. Five patients initially diagnosed as ADEM/CIS were further diagnosed as MS during follow-up. First line treatment with steroids was generally sufficient for complete remission of onset symptoms.