Odontogenic myxoma (OM) is a non-metastasizing neoplasm of mesenchymal origin, arising in the tooth-bearing areas of the jaws. When regarding the whole spectrum of differential diagnoses for osteolytic jaw lesions, OM constitutes a benign tumor rarely located in the maxilla. Radiographically, displacement of teeth and frequent involvement of the sinus will be found with advanced maxillary OM. The tumor can be removed by means of several techniques, ranging from conservative measures to extended surgical procedures that differ according to type of bone resection and reconstruction of the defect. This report documents 10 years of follow-up in a patient suffering from a Type IV lesion of maxillary OM; with a radiographically proven growth extending into the right maxillary sinus, the patient underwent a segmental maxillectomy. After a tumor-free period of 5 years, the alveolar ridge splitting technique (ARST) was modified to insert dental implants into the horizontally deficient alveolar ridge of the maxilla, and final rehabilitation by means of a conditionally removable prosthetic reconstruction followed. In this paper, the most striking clinical signs of OM with which the dentist should be familiar are reviewed, and we discuss the advantages of segmental maxillectomy in case of an OM, along with the possibility of using ARST, aiming at prosthetic rehabilitation by placement of dental implants in cases of moderate alveolar ridge deficiency after tumor resection.