Background: The foci of distant metastasis from extramammary Paget's disease (EMPD) are the lung, liver, truncal bones, vertebrae, and brain. However, skull metastases have not been reported.
Observations: The authors treated a patient with calvarial and skull base metastases from EMPD who had undergone wide local resection of EMPD 8 years before, and they report his clinical course.
Lessons: Because EMPD with distant metastasis is fatal, it should be recognized that EMPD can metastasize to the skull even when it seemed to be in remission for several years.
Keywords: calvarial bone; extramammary Paget’s disease; metastasis; skull.