We report herein a case of tethered cord syndrome with recurrent ulcerations of the skin in a 17-year-old girl. The patient presented an asymptomatic punched-out deep ulcer on the right buttock and right heel. The lesions were noticed as bullae on the sites, which developed into ulceration. At birth, a reddish nodule was noted on the lower back, which was excised when she was 3 years old at a private clinic. Neurological examination revealed hypoesthesia below the LS dermatome. We suspected a simple neurotrophic ulcer which was treated with zinc oxide dressing and oral antibiotics. 1 month later, the ulcers had healed, but tense bullae had developed on the same right buttock. Spina bifida with intraspinal lipoma was revealed by plain X-ray film and magnetic resonance imgaing. Tethered cord syndrome was diagnosed, and treated with a surgical excision of the lipoma, and repair of the dura.