The authors report two cases of cervicomedullary decompression of foramen magnum (FM) stenosis in children with achondroplasia using intraoperative computed tomography (iCT). A 14-month-old girl with myelopathy and retarded motor development, and a 10-year-old girl who had already undergone incomplete FM decompression was presented with myelopathy. Both patients underwent decompressive sub-occipitalcraniectomy and C1 laminectomy without duraplasty using iCT. It clearly showed the extent of FM decompression during surgery, which finally enabled sufficient decompression. After the operation, their myelopathy improved. We think that iCT can provide useful information and guidance for sufficient decompression for FM stenosis in children with achondroplasia.