This report reviews the recent contributions to the field of pathologic nystagmus. The classification and nomenclature of nystagmus with onset in infancy is controversial. Because there are differences in nystagmus forms between patients with idiopathic nystagmus and those with nystagmus associated with afferent sensory defects, a distinction between these two nystagmus types is proposed. The distinctions are also helpful for clinicians, because these separate entities imply different diagnostic evaluations and visual prognosis. Recent studies have confirmed that periodic alternating nystagmus is detected more easily if the patient is evaluated for a longer time period and occurs more commonly than previously thought. Psychophysical investigations indicate that extraretinal signals play an important role in suppression of oscillopsia in infantile nystagmus. Genetic analysis recently has allowed identification of genes of X-linked idiopathic nystagmus and achromatopsia.