Lipomyelomeningocele (LMM) as a cause of tethered cord syndrome (TCS) commonly presents in childhood in the lumbosacral spine. Patients frequently present with cutaneous manifestations, progressive neurological deterioration, bladder dysfunction, and intractable pain. Early surgical intervention with untethering is recommended for symptomatic patients. We report an unusual case of a woman who presented with a subcutaneous lump, pain, and neurological decline found to have a cervicothoracic LMM. The patient underwent laminectomy and subtotal resection of the mass; seventeen years later she was confined to a wheelchair with severe neurological decline ultimately requiring three additional attempts at surgical excision and repair. This case emphasizes the need for early recognition of and intervention in adult patients with LMM.