Spinal extradural arachnoid cysts (SEACs) are uncommon spinal lesions that may cause myelopathy, most frequently in the 2nd decade of life. There are multiple theories of their pathogenesis, and associated entities include spinal trauma, spina bifida, and the lymphedema-distichiasis syndrome. The authors report the case of an otherwise healthy, developmentally normal 13-year-old boy who presented with multiple SEACs. Upon further neuroimaging workup, he was found to have an asymptomatic retrocerebellar arachnoid cyst, cavum septi pellucidi, and cavum vergae. Three contiguous but separate spinal cysts were identified intraoperatively, and they were completely excised with closure of the dural defects. The patient recovered motor and sensory function of the lower extremities. This collection of uncommon neuroimaging findings provides important clues to the pathogenesis of the disease and guides the optimal management of patients with SEACs. The unusual presentation of SEACs, together with uncommon midline abnormalities, provides further evidence of their congenital, midline origin. Therefore, it is reasonable to pursue imaging of the brain in atypical cases of SEACs.