A 65-year-old woman developed freckling on her skin from axillary to inguinal regions at age 40. At age 55 and 63, she had respectively, a subcutaneous tumor from her right buttock and a left acoustic tumor removed, in both cases, histopathological analyses diagnosed a schwannoma. At age 65, she was admitted to our hospital for a detailed examination of an abdominal mass previously detected by MRI.