Vesiculo-bullous lesions as well as the late onset are extremely rare features of Darier disease (DD) making positive diagnosis more difficult. Its association with polycythemia vera prompts to look for signs suggestive of malignant hematological transformation. We report a case of a 62-year-old patient, followed in internal medicine since the age of 58 for polycythemia vera. She presented for pruriginous vesiculo-bullous dermatosis evolving for 4 years. On clinical examination, papulo-vesicular plaques were found involving all the folds: inguinal, axillary, inter-gluteal, neck and body with palmo-plantar blisters and pustules. Examination of the toe nails showed alternating red and white longitudinal bands. Abdominal examination showed moderate splenomegaly. Skin biopsy revealed focal acantholysis with supra-basal clefts, dyskeratosis with the presence of round bodies and grains compatible with Darier's disease. A paraneoplastic assessment was made including a thoraco-abdomino-pelvic CT scan, a breast ultrasound and mammography with cervico-vaginal smear, a CT scan of the cavum and a pancreatic MRI, which did not show any progressive malignant process. The patient was put on oral retinoids at a rate of 0.5 mg/kg per day and topical calcipotriol associated with bloodletting to reduce the hematocrit level. The evolution was marked by clinical improvement. The patient was followed in the hematology department and showed no acute leukemia or myelofibrosis or other neoplasms after a 2-year follow-up. We report a case of late DD in a woman followed for polycythemia vera, characterized by a rare vesiculo-bullous presentation and whose causal link remains to be determined.