The occurrence of intradural spinal teratomas in association with spinal dysraphism is uncommon and even rarer is pulmonary differentiation in a teratoma. We report a case of spinal dysraphism, duplication of lumbosacral spine, split cord malformation (SCM I), and meningomyelocele in an 8-month-old child. Excision of meningomyelocele and detethering of cord was done. Pathologic examination revealed a mature teratoma with pulmonary differentiation. To the best of our knowledge this is the second case of spinal teratoma with pulmonary differentiation.