Introduction In 2013, a rare early complication following cervical decompression the so-called "white cord syndrome" (WCS) was described for first time. This designation was given on the basis of the postoperative appearance of intramedullary hypertense areas in T2-MRI, resulting in devastating neurological damage. To our knowledge, only three cases of WCS have been published; we hereby present the fourth case, but the first one with late-onset presentation of this syndrome. Case presentation A 79-year-old male patient with Nurick grade 3 CSM was referred to our institution. He had already had a double-level C4-C6 anterior cervical decompression and fusion (ACDF) 2 years ago in another institution. The patient underwent posterior decompression from C3 to C6 plus C2-C7 lateral mass screw fusion. Within the first 24 h following surgery, he gradually developed C6 incomplete paraplegia (ASIA B). Cervical MRI disclosed a hypertensive signal in T2weighted sequences at C6-C7 levels and the diagnosis of WCS was suspected. Revision surgery was made 30 h following our first surgery, with wider posterior decompression accompanied by intravenous methylprednisolone. The patient's neurologic status was improved, but the final neurologic outcome was worse (Nurick 4) than the preoperative status and subsequently did not change at all. Discussion To the best of our knowledge, this is the first report of a late-onset WCS and the fourth case of WCS per se. Spine surgeons should be aware of this rare but serious complication. We highlight possible risk factors and review the literature on the hypotheses about the pathophysiology of WCS.