Vertigo is a common principal complaint among patients seen by primary care physicians, neurologists, and otolaryngologists. The most common causes of recurrent episodes of vertigo are benign inner ear disorders, but central nervous system disorders must be excluded. Several common vertigo syndromes are now known to be familial, a feature distinguishing them from other common causes of vertigo. Familial vertigo has been recognized in patients with isolated recurrent attacks of vertigo, genetic deafness syndromes, and in patients with neurological disorders. Although susceptibility loci and mutations have been identified, genetic heterogeneity is common in all familial vertigo syndromes. Research in these disorders has advanced the understanding of vertigo pathophysiology but much remains to be known. This article provides an overview of the clinical features, genetic analyses, and treatment strategies for these disorders.