摘要Primary congenital lymphedema(PCL)is a rare lymphatic disorder that typically presents in early life and is usually managed conservatively.In severe neonatal cases,however,complications such as skin necrosis and coagulopathy can occur.We report the case of a term male infant with prenatally diagnosed vascular malfor-mations who presented with progressive limb swelling,hemorrhagic skin necrosis,and systemic signs of infec-tion.Laboratory findings revealed severe thrombocytopenia and coagulopathy,consistent with Kasabach-Merritt-like phenomenon.The patient underwent staged surgical management,including limb decompression and debridement,followed by negative pressure wound therapy(NPWT),application of an acellular dermal matrix(ADM),and full-thickness skin grafting.The combined use of ADM and NPWT promoted rapid granulation tissue formation and neovascularization,resulting in stable wound coverage and successful limb preservation.Follow-up at 12 months showed excellent graft take,improved limb function,and no recurrence of necrosis or infection.This case highlights the potential role of integrating NPWT and regenerative scaffolds in managing complex neonatal wounds associated with PCL and Kasabach-Merritt-like phenomenon.To our knowledge,this is the first report describing such an approach in a neonate,offering a viable limb-salvaging strategy in otherwise life-threatening presentations.