Background: An angiomyolipoma is a common benign renal mesenchymal tumor but rarely may be found extrarenally. Extrarenal angiomyolipoma (ERAML) arising in the pelvic retroperitoneum is very rare. Case: A 45-year-old multiparous woman presented with a cystic pelvic mass and was misdiagnosed as having an ovarian tumor. Intraoperatively, a retroperitoneal cystic tumor was found and removed. A hysterectomy was necessary in order to remove the mass. Results: Histopathologic examination revealed the cystic mass to be an angiomyolipoma. The patient's postoperative course was uneventful. Postoperatively, this patient is being followed. Conclusions: ERAML should be considered in the differential diagnosis of tumors arising from the pelvic retroperitoneum. Cystic degeneration might occur in ERAML. (J GYNECOL SURG 32:303)
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