A woman in her 50s presented with a 1-year history of vulval soreness and dysuria. She had a medical history of diabetes insipidus and lymphangioleiomyomatosis of the lung diagnosed 25 and 8 years ago, respectively. On examination, there was swelling of the labia majora and loss of the normal architecture, with absence of the labia minora and tethering of the clitoral hood resulting in burial of the glans clitoris. In addition, there were scattered ulcers in the interlabial sulci (Figure, A). A diagnostic biopsy was obtained (Figure, B and C).