Knowledge of the role and hazards of transoral surgery has expanded rapidly, but the application of this technique in children has been limited. To assess its usefulness, 27 pediatric patients who underwent transoral surgery between 1985 and 1994 were studied. Transoral surgery was performed for irreducible anterior neuraxial compression at the craniovertebral junction caused by basilar impression, atlantoaxial subluxation with pseudotumor, or chordoma. The patients ranged in age from 3 to 17 years. Symptomatic presentation varied widely, but 89% had significant neurological deficits before surgery. No patient with normal strength deteriorated after surgery. Of the 16 patients with a preoperative motor deficit, nine improved rapidly, three were unchanged, and four significantly worsened in the perioperative period. Those with mobile atlantoaxial subluxation were most vulnerable to surgically related neurological morbidity. Twenty-four patients were alive for long-term follow-up study (average 5.7 years, range 1-9.2 years). Of those with preoperative weakness, nine improved one Frankel grade, four remained the same, and one deteriorated from Frankel Grade D to C. Swallowing and speech worsened in five patients; this occurred only after resection of lesions above the foramen magnum (p<0.05) when rostral pharyngeal disruption resulted in velopharyngeal dysfunction. This study, unlike previous reviews of pediatric transoral operations, leads the authors to suggest that although transoral surgery can be effective, it also carries a significant risk of neurological injury in patients with symptomatic spinal cord compression and it is also associated with long-term swallowing and speech difficulties.