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Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm occurring mostly in infancy or at birth. 1 In most cases, the lesion develops in soft tissues, consisting of an ill-defined cutaneous, red to purple indurated painful tumour. It may be complicated by Kasabach-Merritt phenomenon (KMP), characterised by severe thrombocytopenia and coagulation activation and rapid enlargement of the lesion. 1,2 Contiguous partial bone involvement is frequent; however, cases of deep locations and even bone location sparing the skin are much rarer. Furthermore, late onset is described in older children, but is rare. 3,4 Atypical features may lead to diagnosis delay. We report two cases of late-onset bone KHE. Those observations illustrate the difficulties in establishing the diagnosis and highlight the dramatic efficacy of sirolimus treatment in this condition.

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