Abstract
Kaposiform hemangioendothelioma is considered a benign vascular tumor, with locally invasive characteristics and histological similarity to Kaposi sarcoma. It is related to complications with varying degrees of morbidity and mortality. The definitive diagnosis is made by histological examination and the treatment course can be either surgical or drug therapy, which varies according to patients clinical indication. The aim of the research is to publish a case of Kaposiform Hemangioendothelioma admitted to a pediatric hospital in Santa Catarina, in 2022. Clinical, epidemiological and laboratory data were obtained by consulting the patients medical record; the reported case data were compared with what is described in the current literature about the disease, discussing their similarities and differences and, finally, confirming the diagnosis, through the anatomopathological exam. Despite the fact of little knowledge between physicians and pediatricians, Kaposiform Hemangioendothelioma is a disease of relevance in the medical field, since its differential diagnoses are the most common vascular tumors of childhood, infantile hemangioma and congenital hemangioma. The purpose of the study is to describe a Kaposiform Hemangioendothelioma case and alert health care professionals the recognition of diseases signs and symptoms and the need to carry out a differential diagnosis in such patients.