Background: Scoliosis is a common finding in children with Chiari I malformation and syringomyelia. The development of scoliosis associated with a Chiari malformation typically has been ascribed to the presence of syringomyelia. Decompression of the hindbrain hernia will often alleviate the syringomyelia and, in many cases, stabilize or reverse the scoliosis. Case report: We report a child with Chiari I malformation and scoliosis who presented with Valsalva-induced headache/neck pain. MRI revealed no syringomyelia or hydrocephalus. Radiographs revealed that her scoliotic curvature was approximately 13° and was a single levoscoliotic curve. This patient underwent a posterior fossa decompression with duraplasty for her Valsalva-induced pain. Postoperatively, she had resolution of her pain and there has been no progression of her scoliosis at 3 years follow-up. Prognosis: Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible. © Springer-Verlag 2006.