Introduction: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignancy with tendency for local invasion and recurrence. The tumor almost exclusively occurs in children, especially in infants. Intracranial KHE are extremely rare with only two cases reported in the literature.
Report: We report the clinical and pathological features of this rare tumor arising from basitemporal region in a 21-month child. Our case did not present with Kasabach-Merritt phenomenon. Histopathological examination confirmed the diagnosis of KHE.
Conclusion: KHE should be considered in the differential diagnosis of intracranial extra-axial neoplasm in children, and histopathological examination plays an important role in distinguishing KHE from its morphologic mimics. It is essential to diagnose KHE due to its locally aggressive nature.
Keywords: Basitemporal; Histopathology; Intracranial; Kaposiform hemangioendothelioma; Rare.