Proteus syndrome is a rare condition that can be loosely categorized as a hamartomatous disorder. It is a complex disorder with multisystem involvement and great clinical variability. At this time, it is unknown how many individuals have Proteus syndrome, since not all cases of Proteus syndrome are documented. We do not know whether anesthetic problems in Proteus syndrome are common or not, but as there is a high incidence of cervical spine involvement, we anticipate significant anesthetic problems. We experienced such a case under general anesthesia for liposuction to lipomatosis in the posterior neck and right upper back, in a 18-year-old boy with Proteus syndrome. We report this case of Proteus syndrome and review the literature on this rare disease.