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Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare, locally aggressive vascular tumors. Although currently classified as separate entities, they are becoming increasingly recognized as a spectrum of the same pathology. There is a well-recognized association with Kasabach-Merritt phenomenon KHE and TA are considered neoplasms of intermediate malignancy because of infiltrative growth, local aggressiveness, and variable prognosis. To date, definitive treatment for these vascular tumors has had limited success.To evaluate the safety, efficacy, and role of vincristine in the treatment of KHE and TA.Case review of patient files and pathology reports at The Children's Hospital at Westmead from 1995 to 2009.Twelve cases with KHE or TA were identified. Seven cases were treated with vincristine. The survival rate in the vincristine group was 100%. Mean age of diagnosis was 30 months (range birth to 9 y). 6 patients were female (85.7%). Mean time of the follow-up was 4 years (range 4 mo to 8 y). Out of the 7 cases treated with vincristine, 3 patients had associated Kasabach-Merritt phenomenon (43%). Complete resolution, regression in size, and improvement in analgesia were found in 1 case (14%), 3 cases (43%) and 2 cases (29%), respectively. Vincristine related side effects occurred in 2 cases (29%).Vincristine is an effective treatment option for KHE/TA. It is associated with a low side effect profile and should be considered as the first-line agent.