DOI: https://doi.org/10.36347/sasjm.2025.v11i05.012

Pages: 446-450

Background: Cutis verticis gyrata (CVG) is a rare dermatologic condition characterized by thickened, folded scalp skin resembling cerebral gyri. Its association with neurofibromatosis type 1 (NF1) is exceedingly rare, with fewer than 20 cases reported worldwide. Case Presentation: A 23-year-old woman presented with progressive CVG and NF1-related neurofibromas involving the scalp, retroauricular region, and lumbosacral area. Surgical excision of redundant scalp tissue and neurofibromas was performed, with histopathology confirming dermal fibrosis and sebaceous hyperplasia. Discussion: We explore the pathophysiological overlap between CVG and NF1, emphasizing the role of RAS/MAPK pathway dysregulation in both conditions. Surgical strategies for CVG in NF1 patients must address vascular preservation and recurrence risk. Conclusion: CVG-NF1 association warrants multidisciplinary management. Scalp reduction surgery provides functional and aesthetic improvement, but long-term surveillance for malignant transformation is critical.