Hemangiolymphangioma is a rare venolymphatic vascular malformation characterized by networks or proliferations of vascular spaces—such as lymphatics, capillaries, veins, or arteries—lined by bland endothelium and embedded in connective tissue stroma. The mesentery is a specialized tissue structure in the abdomen that supports the intestines and contains blood vessels, lymphatic vessels, and nerves that are essential for gastrointestinal function. Mesenteric hemangiolymphangiomas are exceedingly rare in this anatomical location, as vascular malformations more commonly occur in other parts of the body, particularly in the skin, subcutaneous tissues, or organs like the liver, lungs, or brain. The rarity of mesenteric hemangiolymphangiomas is attributable to their unusual anatomical location, the coexistence of both hemangiomatous and lymphangiomatous vascular components, the limited number of documented cases in medical literature, the diagnostic difficulties they pose, and their infrequent occurrence in pediatric populations.This case report details an 8-year-old girl who presented with abdominal distension since past 1.5 years. Imaging modalities, including ultrasonography and CECT of the abdomen, suggested a mesenteric cyst. The patient underwent exploratory laparotomy and excision of the mesenteric cyst. Histopathological analysis of the specimen confirmed the diagnosis of hemangiolymphangioma of the mesentery, further validated by immunohistochemistry. This case highlights the key aspects and histopathological features of this rare condition. Mesenteric hemangiolymphangiomas are intriguing because of their rarity, unique dual vascular origin and diagnostic difficulties. These features make them a valuable subject of study for clinicians and researchers.