Stickler syndrome is an autosomal dominant multisystem disorder with characteristic midface hypoplasia, retromicrognathia, cleft palate and a "moon-shaped" appearance. Progressive myopia and retinal degeneration are frequent. It is estimated that one third of all Pierre Robin patients have Stickler syndrome. Patients with a mandibular hypoplasia like Stickler syndrome present the anesthesiologist considerable problems when mask ventilation or endotracheal intubation is attempted. In this case report the difficulties in anesthetic management of patients with micrognathia and repeated anesthetic courses of a child with Stickler syndrome are presented. It is vital to detect the syndrome in early stages so that adequate counseling and treatment may be given to avoid the potentially irreversible and disabling consequences. © 2006 Mosby, Inc. All rights reserved.