A 41-year-old male presented with a longstanding history of difficulty initiating micturition and lower back pain with recent onset of saddle paraesthesia and bilateral leg pain, which had been associated with decreased sensation on ejaculation and occasional fecal incontinence. There was no history of spinal dysraphism, congenital spinal abnormalities, previous spinal surgery, or lumbar puncture. Clinical examination revealed no evidence of cutaneous abnormalities. Neurological examination was unremarkable. Computed tomography (CT) lumbar spine revealed no bony abnormalities. Magnetic resonance imaging (MRI) confirmed the presence of an intradural, extramedullary lesion at T12/L1, which was causing marked compression of the distal conus. The tumor measured 35 × 14 mm and displayed hyperintense signaling on pregadolinium T1 and low signal on T2 with a small crescentic element of fat on the dorsal superior border Figures and. The surgical management consisted of a laminectomy from T11 to L1 and subtotal excision. At surgery, once the dura was opened the capsule was initially decompressed and a considerable amount of proteinaceous fluid was drained. Thereafter, the majority of the capsule was excised, however, the base of the tumor was found to be densely adherent to the distal conus. It was decided that this could not be safely dissected from the conus and therefore a thin rim of capsule was retained. Postoperatively, the patient developed transient urinary retention, however, he went on to make a good recovery with resolution of his back and leg pain. His bladder function improved although he does report occasional fecal incontinence. Histopathologic examination of the lesion demonstrated elements of endoderm, mesoderm, and ectoderm with fragments of degenerate keratin, cystic spaces lined with stratified squamous epithelium, adipose tissue, disorganized neural tissue as well as mucus secreting epithelium Figure and. There were no immature elements or malignant cells seen. Postoperative imaging at one year confirmed successful decompression of the conus and no evidence of tumor recurrence Figures and.