Background: Kaposiform hemangioendothelioma (KH) is an uncommon tumor in infants and children. The typical clinical presentations include ill-demarcated, red to purple, indurated plaque and are frequently complicated by the Kasabach-Merritt phenomenon (KMP), a condition of severe thrombocytopenia and consumptive coagulopathy. It might be misdiagnosed as infantile hemangioma at birth, leading to a delay in delivering optimal treatment. Therefore, imaging differential diagnosis of KH should be included in pediatric patients presented with atypical vascular lesion for timely management. Case Presentation: We present a case of a 2-month-old male child, born with right lower limb hypertrophy and bluish discoloration, initially diagnosed as a hemangioma. Magnetic resonance imaging diagnosis was suggestive of KH complicated with KMP in correlating with clinical history. Therefore, optimal treatment is possible at a time if correct diagnosis is made early. Conclusion: KH should be retained as one of the differential diagnoses in pediatric patients presenting with vascular tumor. Imaging could help in reaching the diagnosis without invasive surgical interventions in order to initiate proper treatment.

Keywords: Kaposiform hemangioendothelioma, Kasabach-Merritt phenomenon, vascular tumor