Perivascular epithelioid cell tumors (PEComa) are rare neoplasms defined as mesenchymal tumors with distinctive histology and immunohistochemistry. They tend to occur in middle-aged patients and mostly in women. The tumours cells show an association with the walls of blood vessels. Most PEComas are benign although a proportion of these tumours behave aggressively. Case report of 47-years-old woman with retroperitoneal mass and laparoscopic resection that pathological anatomy showed retroperitoneal tumour compatible with PEComas. We describe the main characteristics of PEComas as well as their therapeutic approach.