Kasabach – Merritt syndrome was firstly described in 1940. It’s encompasses a triad of capillary hemangioma, thrombocytopenia and consumptive coagulopathy. Kasabach – Merritt syndrome develops on kaposiform hemangioendothelioma or tufted angioma, confirmed by anatomopathologic analysis which sampling is sometimes difficult to achieve because of bleeding. Consensual care is not established but corticotherapy treatment is unanimous, alone or associated to interferon α 2a or 2b, vincristine, the surgical removal of the mass is controversial.

We report a case of a 5-day old male newborn, with a vascular mass aspect to right forearm who developed on the second day of life an important gastro-intestinal hemorrhage, reason on transfer in teaching university hospital of Lubumbashi. Two complete blood transfusions has been administered to him and biological sampling showed normocytic anaemia, afibrinogenaemia, a severe thrombocytopenia, a disturb of blood test coagulation evoking the Kasabach – Merritt syndrom. After many discussions and consultations, a biopsy was achieved and a diagnosis of tufted angioma has been retained. In the context of the work up of spread, a chest radiography and abdominal ultrasonography were realized without anomalies. Despite the administration of corticotherapy associated to vitamin K, the newborn died the fifth day of life in an important bleeding chart due to consumption coagulopathy.