In this case report we describe a boy with developmental delay and a diffuse large B-cell lymphoma in whom we identified a de novo germline 2p16.3 deletion (134.1 kb), including MSH6 and part of FBXO11. The developmental delay can be explained by the FBXO11 deletion [10, 11]. Here, FBXO11 is linked to diffuse large B-cell lymphoma.