AbstractA 51‐year‐old gardener developed malignant hemangioendothelioma in a lesion of discoid lupus erythematosus (DLE) after repeated occurrence of squamous cell carcinoma and keratoacanthoma. The hemangioendothelioma arose in the same DLE lesion where squamous cell carcinoma had appeared. It was treated with surgical exision followed by intraveneous dimethyltriazenoimidazole carboxamide (DTIC). He has not shown any signs of recurrence for a follow‐up period of 45 months. This is the first report, to our knowledge, of the occurrence of malignant hemangioendothelioma in a DLE l