According to updated ISSVA 2018 classification, kaposiform hemangioendothelioma (KHE) is related to locally aggressive tumors. In most cases – approximately in 79%, the first clinical signs appear in neonatal period. When located in liver, this symptomatic tumor tends to clinically-cacoethic presentation with life threatening complications development and high death rate. Among such complications Kasabach–Meritt syndrome, intraabdominal bleedings, cardiac failure, and respiratory disorders should be mentioned. Numerous treatment options of KHE and its complications are being used, but no standardized approach exists. To verify the final diagnosis immunohistochemical analysis of tumor tissues must be performed for endothelium proliferation markers, such as CD-31 and CD-34. Differential diagnosis of hepatic vascular tumors is complicated because the biopsy is not performed in general, as there is a high risk of massive bleeding or uncontrolled tumor growth provocation. The success of KHE treatment depends on pediatric surgeons’, pediatricians’, and neonatologists’ knowledge on this pathology, on careful analysis of clinical presentation and diagnostic findings, especially of imaging methods like computer tomography (CT) or magnetic-resonance imaging (MRI). The article highlights the clinical case of complex treatment of hepatic Kaposiform hemangioendothelioma in a newborn with vincristine administration, that allowed to perform the surgical resection of vascular tumor. The current state literature review is presented. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies.