Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of childhood, often associated with Kasabach-Merritt phenomenon (KMP), a life-threatening coagulopathy with thrombocytopenia. In the described case, the child had multifocal involvement of several organs in the abdominal cavity, namely the head of the pancreas, liver, bile ducts and duodenal papilla. Contrast-enhanced ultrasound (CEUS) showed the characteristic inhomogeneous hypoenhancement of KHE during the arterial and portal phases, followed by complete washout and absence of enhancement in the late phase. After the failure of several treatment attempts, a pancreaticoduodenectomy was performed, which was successful. This case highlights the importance of surgical resection in KHE when possible and safe. Recognizing the specific features of CEUS is crucial for accurate diagnosis and avoiding diagnostic errors.