As a vascular malformation, venous angioma in the spinal cord is extremely rare. To our knowledge, there are only five case reports in the literature, and it has not been previously reported in the pediatric age group. In this paper, we report on a 3-year-old patient who presented with progressive weakness in his left upper limb. Spinal magnetic resonance imaging (MRI) revealed an epidural cystic mass at the C6-T2 level. The lesion was diagnosed as venous angioma after total removal with laminectomy. Postoperatively, the patient remained symptom free, and no tumor recurrence was confirmed based on MRI at the time of the 18-month follow-up. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all six reported cases were reviewed. Venous angiomas should be included in the differential diagnosis of spinal cystic lesions in children. A definitive diagnosis is difficult based on MRI alone. This rare lesion is amenable to surgery, and gross total removal (GTR) is usually achievable due to a well-demarcated dissection plane. A good clinical outcome after GTR can be expected.