This article presents a child with Chiari I malformation and cutis marmorata telangiectatica congenita and reviews the medical literature regarding Chiari I malformation and other cutaneous disorders. In addition to cutis marmorata telangiectatica congenita and Chiari I malformation, our patient exhibited hemihypertrophy, Tourette's syndrome, scoliosis, obsessive-compulsive disorder, and seizures. Other associated findings seen in patients with Chiari I malformation include basilar invagination, Klippel-Feil syndrome, atlantoaxial assimilation, scoliosis from an underlying syrinx, and hydrocephalus.