Maffucci's syndrome is a congenital disease characterized with enchondromatosis and multiple soft tissue haemangiomata due to neuro-ectodermal dysplasia. Maffucci's syndrome is a rare clinical condition that presents difficulties concerning its anesthetic management. In this presentation, our anesthesia approach on the patient with Maffucci's syndrome with multiple haemangiomata along the upper airways requiring intralesional sclerotherapy is described.

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