Background: Squamous cell carcinoma (SCC) arising in chronic lymphedema is rare; only nine cases have been reported.
Objective: To present the evolution of SCC in chronic lymphedema.
Methods: Case report and literature review.
Results: The tumor was treated by wide excision and covered by a skin graft.
Conclusion: In most of the other reported SCC cases in lymphedema, there are additional factors for carcinogenesis. There is no additional carcinogenic factor except for chronic lymphedema in our case. This strongly supports that lymphedema itself is one of the carcinogenic factors for not only angiosarcoma but also SCC.