Background: Venous malformation, formerly designated as cavernous hemangioma, is a rare vascular lesion characterized by significant endothelial cells proliferation, predominantly affecting young females. Diagnosis is challenging due to its low incidence and variety of clinical, radiological, and histological presentations. Objectives: The aim was to review the current scientific understanding of maxillary venous malformation based on the available literature and present an additional rare case of venous malformation of the upper maxilla. Methods: A systematic review was conducted across PubMed, Google Scholar, and Embase databases for studies published between January 1990 and April 2025. Inclusion criteria encompassed meta-analyses, systematic reviews, randomized controlled trials, non-randomized controlled trials, cohort studies, and case reports describing cavernous hemangiomas and venous malformation of the maxilla. All clinical and radiological characteristics were considered. Results: Out of 10,021 studies identified through our database search, 22 met the inclusion criteria, describing 28 (29 with our case) clinical cases of maxillary venous malformation. Conclusions: Maxillary venous malformation presents complex and varied clinical and radiological aspects, which are crucial for preoperative assessment and management. Appropriate measures may be necessary to prevent bleeding complications during lesion removal. To the best of our knowledge, this is the first comprehensive review on venous malformation of the maxilla. In addition, we report an unusual case identified incidentally during implant planning and successfully removed through isolated bone augmentation.