Introduction: Here we are presenting a unique case of malignant triton tumor of the trigeminal nerve in a 4-year-old boy who presented with diplopia and ptosis.
Intervention: Near total excision of the tumor was performed, and adjuvant chemotherapy and radiotherapy were administered.
Results: The patient is in good health and has no evidence of clinical and radiological tumor recurrence for 22 months.
Keywords: Childhood; Cranial nerve; Nerve sheath tumor; Treatment.