1型神经纤维瘤病是一种多系统疾病，主要涉及神经系统和皮肤。1型神经纤维瘤病的并发症包括发育源自神经嵴的恶性肿瘤，如皮肤黑色素瘤。皮肤黑色素瘤在0.1％-5.4％的NF1患者中发现，黑人患者发病率要低得多。我们介绍了一名53岁的女性，已知是NF-1的病例，出现多发性神经纤维瘤。其中一种在12岁时就出现在头皮上。在症状出现前两个月，头皮神经纤维瘤开始迅速增大，并出现多个溃疡。CT扫描显示软组织肿块未附着于骨骼或其他颅骨结构。患者经手术切除，留有安全缘延伸至完整厚度皮肤移植。组织病理学显示肿瘤有自由边缘，初步表明为梭形细胞肉瘤，然后进行免疫组织化学检查结果显示，头皮黑色素瘤。随访表明皮肤移植成功，患者被送往肿瘤科进行进一步治疗。黑色素瘤在NF1患者中较为罕见，但也可能发生。重视这些患者出现黑色素瘤的可能性并进行定期皮肤检查非常重要。 NF1患者的黑色素瘤治疗遵循与非NF1患者相同的指南，根据黑色素瘤类型的不同，其切除边缘可以宽或窄。根据缺陷的大小和位置，可以使用半厚度或全厚度皮肤移植进行缺损的修复。黑色素瘤是一种毁灭性的皮肤恶性肿瘤，任何出现皮肤病变甚至与罕见疾病有关的病变都应引起怀疑。版权所有©2023作者。由Elsevier Ltd。保留所有权利。

Type 1 neurofibromatosis is a multisystem disease, mainly involving the nervous system and skin. Complications of type 1 neurofibromatosis are the development of neural crest derived malignancies such as melanoma of the skin. Skin melanomas have been found in 0.1 %-5.4 % of NF1 patients and it's far less likely to develop in Black people.We present a 53 years old female known to be a case of NF-1 with multiple neurofibromas. One of them in the scalp since the age of 12. Two months prior to presentation, the scalp neurofibroma start to increase in size rapidly and multiple ulcers developed. CT scan showed soft tissue mass not attached to the bone or other cranial structure. The patient underwent excision with safety margins with a full thickness skin graft. Histopathology showed a free edge of the tumor and was initially suggestive of spindle cell sarcoma then immune-histo-chemistry requested the result showed, melanoma of the scalp. The follow-up showed a good take of skin graft and the patients was sent to oncology for further management.Melanoma is a rare malignancy in patients with NF1, but it can occur. It is important to be aware of the possibility of melanoma in these patients and to perform regular skin checks. Treatment of melanoma in NF1 patients follows the same guidelines as for non-NF1 patients, with wide or narrow excision margins depending on the type of melanoma. Reconstruction of the defect can be done with split-thickness or full-thickness skin grafts, depending on the size and location of the defect.Melanoma is a devastating skin malignancy and should be in suspicion for any skin lesion that present even in association with rare diseases.Copyright © 2023 The Author(s). Published by Elsevier Ltd.. All rights reserved.