An unusual case of cholelithiasis in an 18-month-old boy with cervico-
oculo-acoustic (Wildervanck's) syndrome is presented. Our patient had
Duane's retraction syndrome, Klippel-Feil anomaly and congenital deafn
ess. To our knowledge this is the first case in which a probable assoc
iation between cholelithiasis and Wildervanck's syndrome has been reco
rded. On the other hand, the presence of mutual malformations and anom
alies such as scoliosis, ventricular septal defect, ectopic kidney, hy
drocephalus, hypoplastic thumb and growth retardation seems to suggest
that Wildervanck's syndrome is a clinical variant of Klippel-Feil seq
uence.