Introduction. We report a case of unilateral optic disc edema in a paediatric patient and discuss the concerns involved in diagnosis and management of similar cases. Materials and Methods. Female aged 10 years was referred to our clinic due to progressive visual loss of the LE over a few days. Her visual acuities (VA) were RE 10/10, LE 3/10, and she had a relative afferent pupillary defect and decreased colour vision in her LE and normal and painless eye movements. Fundoscopy showed a remarkably swollen disc of the LE, and visual field (VF) examination revealed enlargement of the blind spot and presence of horizontal inferior papillomacular scotoma. Neurological examination, CT of brain and orbits and blood tests were normal. Visual evoked potentials revealed an obstacle in the myelin substance before the optic chiasma of the LE. Results. The patient was treated with intravenous methylprednoslone for 3 days and with oral methylprednizole for 15 days in progressively diminished daily doses. This led to gradual improvement of VA, colour vision, and visual field and resolution of optic disc oedema. Discussion. Concerns that have to be taken into account regarding diagnosis and management of similar cases are related to lumbar puncture indications, treatment with corticosteroids, and appropriate followup.