The etiology of strabismus has long been observed to have a genetic co
mponent. Recent advances in genetic methodology may provide insight in
to the genetic basis for several types of inherited strabismus, includ
ing those associated with genetic multisystem disorders such as Moebiu
s syndrome, Prader-Willi syndrome, craniofacial dysostoses, and mitoch
ondrial myopathies. Inheritance of primary forms of strabismus, such a
s congenital ocular fibrosis, Brown syndrome and Duane syndrome, has b
een reported, but less is known of the defective genetic sites. The ge
netic basis for isolated strabismus that clusters in families, such as
infantile esotropia syndrome, is also not yet known, but new techniqu
es of molecular biology may now permit linkage detection in these fami
lies. By identifying affected families, clinicians will take part in u
nraveling the genetic basis of hereditary strabismus syndromes.