Diagnosis of neoplasms of the optic-chiasmatic region is facilitated by assessment of onset symptoms. They represent the basis for a specific radiologic study, allowing a differential diagnosis between radiologically similar forms. An easy approach to these neoplasms is to separate malignant lesions from non-neoplastic space-occupying lesions. Diagnostic imaging of neoplasms of the optic-chiasmatic region is based on MRI, occasionally combined with CT. Because of its peculiar embryology, the region is the site of numerous neoformations. However, differential diagnosis is ready in most cases. To plan the most suitable surgical management, the relations of the lesions with adjacent anatomical structures should be carefully assessed. More complex syndromes (gliomas in NF1, infundibulopeduncular histiocytosis in eosinophilic granulomatosis, etc.) should also be considered.