It usually lacks a known recurrent genetic abnormality and remains unclassified by currently established tumor entities.[2] Recently, 2 recurrent oncogenic fusion rearrangements, BCOR-CCNB3 and CIC-DUX4 have been described in the literature,[4–10] but reports on infantile ELS/URCS are scarce. The gene discussed is CCNB3; the disease is neoplasm.