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CHONDROPLASIA is an autosomal-dominant, inherited dwarfism syndrome characterized by short stature, macrocephaly, shortening of the extremities, and a cluster of other skeletal abnormalities. This disorder is due to a point mutation in the gene for the fibroblast growth factor receptor on chromosome 4 and affects one in 26,000 live births annually. 3,8,19 Although the gene is inherited in an autosomal-dominant manner, approximately 75% of cases are due to a new, sporadic mutation. 3 When sporadic mutations occur, they are almost always associated with the paternal allele, which is consistent with the association of this disorder with advanced paternal age. Patients with achondroplasia are susceptible to neurological compression at a number of levels along the neuraxis. In the pediatric population, one region commonly affected is the craniocervical junction. Neurological dysfunction may result from compression on the brainstem or upper cervical spinal cord in this setting. The manifestations of neurological dysfunction in this patient population may be subtle and difficult to detect clinically. Patients with achondroplasia are well known to be hypotonic during early infancy, and they achieve motor milestones at a slightly slower pace than do unaffected individuals. 10 In addition, nonskeletal abnormalities may mask or confound the clinical picture. For example, patients with achondroplasia may often suffer from concurrent central and obstructive apnea attributable to neurological compression (central apnea) and upper airway causes such as micrognathia and tonsillitis (obstructive apnea). In this paper we review our clinical experience with the surgical treatment of symptomatic cervicomedullary compression over an 11-year period (1993‐2003) in pediatric patients with achondroplasia. We identified 43 patients from our database who were treated during this period, and our clinical experience with these individuals is presented here.