Background: Therapy of epitheloid angiomyolipomas (eAML) may be challenging, since unlike classical angiomyolipomas this rare subclass of benign mesenchymal angiomyolipomas may present with lymph node metastases, local recurrent disease, and/or systemic metastatic disease in up to 30% of cases., Objectives: We report here for the first time in Germany a case of eAML after successful treatment of malignant melanoma., Materials and Methods: Clinical and histological findings as well as results of the genetic analysis of the angiomyolipoma are presented., Results: A somatic, truncating mutation of the TSC2 gene was found in the angiomyolipoma., Conclusion: The relationship to histologically similar tumor entities are presented and therapeutic options based on the genetic classification are discussed., (© 2021. The Author(s).)