BACKGROUND: Perivascular epithelioid cell tumors (PEComas), make up a family of extremely rare mesenchymal neoplasms, with characteristic morphological, immunohistochemical and molecular findings. Malignant PEComas and gastrointestinal epithelioid angiomyolipoma (E-AML) are especially rare. To the best of our knowledge E-AML have not been found in the breast. The difficulty in determining what constitutes optimal therapy for PEComas, owing to the sparse literature available, led us to report this rare case. METHODS: We report a case of a 44-year-old woman, with a family history of multiple endocrine neoplasia syndrome (MEN) (gastrinoma, medullary thyroid cancer and parathyroid hyperplasia), affected by PEComa located in the kidney, stomach, ileum, liver and breast. RESULTS: The renal, gastric, ileal and mammarian tumors were completely resected, with no evidence of local disease. Liver lesions were biopsied. The morphological and immunohistochemical findings confirm the diagnosis of PEComa. CONCLUSION: On this basis it is difficult to determine if some E-AML are multifocal tumors or metastatic disease.