Hereditary multiple exostoses (HME) generally involves the long tube bone or flat bone. Spinal involvement is rare, particularly in young children. We report two cases of children with HME who have myelopathy because of cervical spine involvement and then review the literature to clarify the clinical characteristics of spinal cord HME involvement in young children. In our cases and previously reported cases, neurological deficits remained in cases with acute onset, but other cases with slow onset showed almost complete recovery after surgery. This suggests that the assessment of spinal lesions before trauma is very important.