Symptomatic occipital lobe epilepsy is increasingly recognized among patients with partial-onset seizures. Although traditional clinical and electroencephalographic criteria had defined occipital lobe epilepsy in the past, new neuroimaging techniques and the recognition of specific syndromes associated with occipital lobe epilepsy have improved the diagnosis and management of these patients. These syndromes include, among others, lesional occipital lobe epilepsy (congenital vs. acquired), MELAS, and epilepsy with bilateral occipital calcifications. The diagnosis of symptomatic occipital lobe epilepsy is improving as functional and structural neuroimaging techniques enable the detection of subtle abnormalities in such patients. This has had a direct impact on the correct classification of patients with benign occipital lobe epilepsy, basilar migraine, and symptomatic occipital lobe epilepsy. The common clinical symptoms, EEG patterns, and neuroimaging findings of these patients are discussed.