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Background  Rapidly involuting congenital hemangioma is a congenital soft tissue tumor, which is difficult to excise completely and rather prone to recur. This atypical tumor combined with capillary-lymphatic-venous malformation was not reported in the literature.    Case presentation  We report an atypical case of a 16-year-old teenager who was born with a mass in his right upper limb. Since there is a recurrence after excision for several times and had a serious impact on daily life, we chose amputation. After the surgery, the patient gained a functional recovery. Two years after the surgery, he had no tumor recurrence.    Conclusions  For this rare tumor with repeated recurrences and significant impact on daily life, we suggest performing amputation at the early stage.

A rare atypical rapidly involuting congenital hemangioma combined with vascular malformation in the upper limb