Background: Involvement of the female genital tract by Darier's disease, an unusual genodermatosis, is uncommon, and the manifestation of the disease in a cervicovaginal smear is exceptionally rare.
Case: A 39-year-old woman had an abnormal Pap smear caused by involvement of the female genital tract by Darier's disease. Cytologic examination showed features consistent with a low grade squamous intraepithelial lesion, but during a biopsy it was found to be vaginal involvement by Darier's disease.
Conclusion: The correct interpretation of cytologic findings is not possible when the diagnosis of Darier's disease is not known since a low grade squamous intraepithelial lesion cannot be ruled out. This case underlines the importance of knowing the patient's medical histoiy in any moment of medical attention.