Objectives The aim of this study was to integrate the available data published on intravascular papillary endothelial hyperplasia (IPEH) into a comprehensive analysis of their clinic, radiographic, and imaginologic features, treatment, and recurrence. Methods An electronic search was undertaken in April 2018 in 3 databases, including cases reports and cases series of IPEH. Eligibility criteria included publications having enough clinical, radiologic, and histopathologic information to confirm a definite diagnosis of the lesion. Results Thirty studies were included in the systematic review with 86 cases of IPEH. The mean age was 46 years—with a predilection for females (55%). Asymptomatic (88%) lesions on the lower lip (39%), with a clinical presentation of firm nodular lesion, bluish, or reddish coloration and mean size of 1.6 cm were the most observed. When intraosseous, the radiographic features were multilocular radiolucent area. Of informed cases, treatment chosen was excisional biopsy (96%) and no recurrence was observed. The diagnostic hypothesis most prevalent was hemangioma, in 27 cases (31%). Conclusions The features presented in this study are more similar to than different from those reported in the literature. The aim of this study was to integrate the available data published on intravascular papillary endothelial hyperplasia (IPEH) into a comprehensive analysis of their clinic, radiographic, and imaginologic features, treatment, and recurrence. An electronic search was undertaken in April 2018 in 3 databases, including cases reports and cases series of IPEH. Eligibility criteria included publications having enough clinical, radiologic, and histopathologic information to confirm a definite diagnosis of the lesion. Thirty studies were included in the systematic review with 86 cases of IPEH. The mean age was 46 years—with a predilection for females (55%). Asymptomatic (88%) lesions on the lower lip (39%), with a clinical presentation of firm nodular lesion, bluish, or reddish coloration and mean size of 1.6 cm were the most observed. When intraosseous, the radiographic features were multilocular radiolucent area. Of informed cases, treatment chosen was excisional biopsy (96%) and no recurrence was observed. The diagnostic hypothesis most prevalent was hemangioma, in 27 cases (31%). The features presented in this study are more similar to than different from those reported in the literature.