Introduction: Orbital schwannoma is a rare pathology, which constitutes approximately 1 to 6.5% of orbital tumors, and can originate from the ophthalmic branch of the 5th cranial nerve or from perioptic sympathetic nerves. Its diagnosis is made by magnetic resonance imaging (MRI) with contrast. The first-line treatment is surgery, and total resection provides a good prognosis. The time of illness is used to evaluate the visual prognosis in these patients.

Clinical Case: A 12-year-old woman, with a 9-year illness, characterized by a progressive decrease in right visual acuity until reaching amaurosis. Brain MRI with contrast shows an isointense tumor on T1, adhered to the medial aspect of the optic nerve sheath, which captures contrast, slightly hyperintense on T2. Total resection of the lesion is performed, and the diagnosis of schwannoma is confirmed by pathological anatomy. A month after surgery, the patient had slightly recovered her vision, without presenting other complications.

Conclusion: Orbital schwannoma is a rare pathology that must be treated surgically as soon as possible to achieve a better visual prognosis for the patient.