Introduction: Neurofibromatosis (NF) is an autosomal dominantly inherited disease, classified as NF type 1 (von Recklinghausen disease) and type 2 (NF2). Palindromic rheumatism can be characterized either by permanent deformity or rapid arthritic or para-arthritic attacks that do not cause radiographic changes. These attacks can continue for hours, days, or weeks. Case Report: A 59-year-old female presented with diffuse pain and joint swelling attacks that had prevailed for one year. Many soft neurofibromas were observed on her body, with skin-colored palpations ranging in size from 0.5 to 5 cm. Brown, mm-sized, axillary freckles were observed on both her underarms. There were five cafe-au-lait spots on abdominal trunk. Diffuse sensitivity and swelling was observed on both wrists. She had thoracolumbar kyphoscoliosis and macrocephaly. Conclusion: Palindromic rheumatism in a NF has rarely been reported.