We report a new case of lymphangioleiomyoma in a 29 years old woman, with antecedents of recurrent spontaneous pneumothorax; she was diagnosed 2 years previously of retroperitoneal hemangiopericytoma, and she has admitted to the hospital because pneumothorax, chilotorax and honeycomb lung pattern, which induce to review the previous retroperitoneal biopsy and confirm to answer with lymphangioleiomyoma. The literature is reviewed.