A 2-month-old infant presented with a cervical intramedullary spinal cord cavernous angioma manifesting as left hemiparesis caused by hematomyelia. Osteoplastic laminotomy of the cervical spine was carried out with subsequent microsurgical excision of the intramedullary spinal cord cavernous angioma. Magnetic resonance imaging revealed no residual tumor. At 25 months after the surgery, she presented no neurological deficit without recurrence and cervical deformity. This case of infantile intramedullary spinal cord cavernous angioma presenting with cervical hematomyelia shows osteoplastic laminotomy of the cervical spine might be helpful to prevent consequent cervical deformity in pediatric cases.