This scoping review focuses on intravascular lobular capillary hemangioma (ILCH), a rare and distinct subset of lobular capillary hemangioma (LCH). This study provides a comprehensive overview of ILCH, delving into its clinical characteristics, origins, pathogenesis, diagnostic methods, treatment options, and outcomes. Despite its rarity, ILCH presents unique diagnostic and management challenges due to its intravascular origin. The review emphasizes the importance of accurate differentiation from other vascular lesions and underscores the need for histopathological confirmation. This article discusses the presentation of ILCH in the reported literature. The pathogenesis remains uncertain, with factors such as trauma, inflammation, hormonal changes, and medications being considered potential contributors. Histopathological features, imaging techniques, and diagnostic tools are discussed, highlighting the distinct histological architecture of ILCHs and the importance of immunohistochemical staining for accurate diagnosis. Surgical excision is the primary approach for managing ILCH due to its potential complications, including superior vena cava (SVC) occlusion and thrombosis. This review concludes by outlining potential directions for future research, including investigating genetic and molecular mechanisms underlying ILCH development, developing targeted therapies, building patient registries for collaborative efforts, and exploring minimally invasive surgical techniques. The importance of long-term patient outcome studies and international collaborations is emphasized to enhance our understanding of this rare vascular anomaly.