Lymphangioleiomyomatosis is a rare disease involving the lung, lymphat
ic trunk and lymph nodes. Sometimes it appears in the retroperitoneum
with or without subsequent development in the lungs. We present a 52-y
ear-old woman with retroperitoneal location of the disease. We discuss
the abdominal CT appearance, the cytologic and immunophenotypic featu
res and a preoperative differential diagnosis is attempted.