The presence of gangliogliomas in combination with a diagnosis of neurofibromatosis type 1 (NF1) is extremely unusual. Here, we present a case of spinal cord ganglioglioma of a child who also had NF1 and attempt to describe the natural history and treatment course of this tumor. Ten years prior to this report, a 16-year-old boy affected by NF1 was diagnosed with an enhancing tumor of the spinal cord that extended from C3 to T1. After radiotherapy was applied, he chose a follow-up of conservative observation. Ten years later, the patient underwent a subtotal resection. A pathological examination revealed a ganglioglioma. We are of the opinion that the malignant transformation of the ganglioglioma in this patient was more likely a natural occurrence rather than a result of radiotherapy. Due to its favorable survival rate, ganglioglioma should be considered when differentially diagnosing an NF1 patient who presents with a spinal cord tumor.