A case is presented in which diffuse lymphangiomatosis resulted in the complete loss of the maxillary dentoalveolar complex and underlying basal bone. The complex investigation and treatment of this patient over a 10-year period is presented, and the importance of a multidisciplinary team approach in providing a functional and esthetic rehabilitation is highlighted. The use of a vascularized bone graft based on the deep circumflex iliac artery and subsequent restoration with an implant-supported prosthesis is described.