Therefore, in line with the fifth edition of the World Health Organization (WHO) classification of bone and soft tissue tumors, in which lesions previously referred to as arteriovenous malformation in soft tissue, are renamed as “arteriovenous malformation/hemangioma” based on the presence of MAP2K1 gene mutations3 we propose to refer to the vascular lesions reported here as “vascular malformation/hemangioma with EWSR1-NFATC2 rearrangement.” Here, EWSR1 is linked to arteriovenous hemangioma/malformation.