A 36-year-old Japanese male, who 7 years previously had been diagnosed as having an ependymoma in the left parietal region, had received surgery, chemotherapy and radiotherapy. He later developed a rapid growth of multiple skin lesions on his back and extremities, which coincided with a regrowth of the tumor. Postmortem examination revealed that the ependymoma showed anaplastic transformation and necrosis; however, no malignancy was observed in the extracranial organs. The skin lesions were histologically diagnosed as seborrheic keratoses. We have, therefore, diagnosed the patient as having Leser-Trélat sign associated with anaplastic ependymoma.