Mowat-Wilson syndrome is a genetic disease caused by heterozygous mutations or 
deletions of the zinc finger E-box-binding homeobox 2 (ZEB2) gene. The syndrome 
is characterized by typical facial features, moderate-to-severe mental 
retardation, epilepsy and variable congenital malformations, including 
Hirschsprung disease, genital anomalies, congenital heart disease, agenesis of 
the corpus callosum, and eye defects. The prevalence of Mowat-Wilson syndrome is 
currently unknown, but it seems that Mowat-Wilson syndrome is underdiagnosed, 
particularly in patients without Hirschsprung disease. We report here the first 
Egyptian case of Mowat-Wilson syndrome who was conceived by intracytoplasmic 
sperm injection. The patient manifested bilateral sensorineural hearing loss--a 
new feature not previously reported in cases of Mowat-Wilson syndrome. This 
report describes the first Egyptian patient of Mowat-Wilson syndrome who was 
conceived after intracytoplasmic sperm injection, and provides a new evidence 
for the inclusion of deafness among the congenital defects of the syndrome.