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Vascular lesions are the most common congenital and neonatal abnormalities. About 60% of anomalies occur in the head-and-neck region due to its complex vascular anatomy. There is confusion in the descriptive terminology for the vascular entity and eponyms. Thus, appropriate classification based on clinical examination and imaging is essential for proper diagnosis and treatment. Thus, the aim of this article is to report a case of venolymphatic malformation with comprehensive knowledge about classifications, pathogenesis, clinical presentation, and its management.