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We present a rare case of optic neuropathy due to anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL) with optic nerve infiltration in a 12-year-old girl who presented with acute unilateral vision loss, diplopia, and headache after two prior hospitalizations at an outside facility for disk edema. She had a presumptive diagnosis of neurosarcoidosis and empiric treatment had been initiated with high-dose corticosteroids. Ongoing worsening of vision prompted presentation at our facility.

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Intracranial Hypertension (ICHT) is identified as the elevation of Cerebrospinal Fluid (CSF) pressure in patients devoid of any underlying causes. Optic Neuritis (ON) is not typically seen as a complication of ICHT, and patients diagnosed with concurrent manifestation of both these disorders usually have some identifiable underlying cause. In this report, we highlight the clinical and para-clinical findings in two unrelated children presenting with high CSF opening pressures and Optic neuritis in the absence of any identifiable neurological or immunological cause.

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Optic neuritis (ON) is defined as an acquired disorder of the optic nerve that may be associated with demyelinating diseases or infectious or inflammatory processes. In children, the manifestation of this condition differs from that in adults, where it typically presents with bilateral papillitis subsequent to a preceding viral illness. Nonetheless, the main concern for practitioners is the possibility of its conversion to multiple sclerosis (MS).

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