Abstract

Nance-Horan Syndrome is a very rare genetic disorder that may be evident at the
time of birth as a congenital condition affecting eyes and teeth. As this syndrome is rare it is
probably under diagnosed. In addition to dental anomalies and eye abnormalities, the patient
may suffer from intellectual impairment if the patient is a male because it is inherited as an Xlinked
trait. We report a case of this male predominant syndrome in a 12 year old female who
presented to us with unusual dental morphology and congenital cataract.

Key words: Nance-Horan syndrome; Genetic disorder; ocular anomalies; Dental defects.