There are many lesions that can present as an elevation of the optic disc. Since each of them has different ophthalmic and systemic significance, it is important for the ophthalmologist to recognize them and to plan strategies of management when necessary. Important inflammatory and benign congenital conditions should be differentiated from malignant neoplasms. This review covers the differential diagnosis of lesions that can cause an elevation of the optic disc. It includes selected congenital lesions, inflammatory conditions, non-inflammatory non-neoplastic disorders, benign hamartomas, and primary and secondary malignant neoplasms.