The assessment of a patient with binocular vertical diplopia begins with a thorough history and neuro-ophthalmologic examination. The neuro-ophthalmologic examination includes observation for a compensatory head, face, or chin position; ocular ductions and versions in the nine cardinal positions of gaze; the three-step test; the double Maddox rod test; indirect ophthalmoscopy to observe the location of the fovea in relationship to the optic nerve head to determine cyclodeviation; and the forced ductions test. Binocular vertical diplopia may be due to supranuclear processes, ocular motor nerve dysfunction, neuromuscular junction disease, diseases of eye muscle, mechanical processes causing vertical eye misalignment, and even retinal disease. In this article, the differential diagnosis of these processes is outlined.