BACKGROUND: A variety of complications after FMD with or without duraplasty for Chiari malformation have been described. Although cerebellar ptosis through the dural opening and pseudomeningocele are well-known rare complications of this procedure, spinal cord herniation manifesting as pseudomyelomeningocele formation has not previously been cited in the literature. In this report, we present a case of delayed pseudomyleomeningocele after FMD. CASE DESCRIPTION: A 22-year-old man presented with progressive spinal cord compression symptoms 7 years after undergoing FMD for Chiari malformation. Craniocervical MRI of the patient revealed pseudomyelomeningocele at C1 level together with cord distortion. The patient underwent untethering surgery for spinal cord adhesions; neural tissue was freed microsurgically from the adjacent structures and duraplasty was performed with synthetic graft. In the early postoperative period, his symptoms resolved moderately. CONCLUSION: Neural tissue shift including the spinal cord through the dural opening may occur after a large posterior fossa decompression without duraplasty for Chiari malformation. Postoperative inadequate CSF circulation due to arachnoid scarring around the decompression area may facilitate this type condition. Early diagnosis of such a complication is of vital importance to prevent serious neurologic deficits, and release surgery with proper duraplasty and also restoration of CSF circulation is the choice of treatment.