Spina Bifida occulta usually presents with some cutaneous stigmata e.g. hair patch, sinus, lipoma, hyperpigmented skin and very rarely a congenital tail. A congenital tail may and may not be associated with spina bifida occulta and tethered cord. A four month old male child presented with congenital tail which was associated with spinal dysraphism and caused tethering of the cord itself. The tail and tethering lesion were excised successfully.