Shprintzen-Goldberg syndrome is a rare connective tissue disorder characterized 
by marfanoid habitus and additional dysmorphic stigmata. Craniocervical 
anomalies occur in fewer than 30% of cases. Serious vertebral instability can 
also occur, albeit rarely. The authors report on the first patient treated with 
surgical fusion at the craniocervical junction because of a C-1 dysplasia and 
severe instability. The skeletal and cardiovascular anomalies that can pose 
additional problems for surgical treatment and perioperative care are discussed 
in detail.