<p>Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm of intermediate malignancy, typically diagnosed in infancy. However, it may mimic high-flow vascular malformations, resulting in misdiagnosis and delayed treatment. We describe a 10-year-old girl with a progressively enlarging swelling on the medial aspect of the right leg, initially diagnosed as an arteriovenous malformation (AVM). She underwent sclerotherapy and two sessions of staged embolization, which were complicated by inflammation, abscess formation, and a chronic non-healing ulcer. She underwent en-bloc wide local excision of a highly vascular lesion, followed by split-thickness skin grafting. Histopathological examination confirmed KHE. The patient had an uneventful postoperative recovery, with satisfactory graft uptake and no recurrence at three-month follow-up. This case illustrates the diagnostic challenges of KHE in older children, particularly when clinical and radiological findings overlap with AVMs. Embolization, though useful for debulking, failed to achieve durable control and complicated the clinical course. Histopathology remained the cornerstone of diagnosis. Multidisciplinary management enabled safe resection and functional wound coverage. KHE may closely mimic vascular malformations, delaying definitive therapy. Early consideration of KHE in atypical lesions, combined with a multidisciplinary approach, can facilitate timely diagnosis, and optimize outcomes.</p>