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The synchronous presentation of two primary malignancies is rare, even more so when two malignancies coexist within the same location as in the case of a collision tumor. While collision tumors involving colonic adenocarcinomas have been reported, those involving an indolent B-cell lymphoma are somewhat rare. In addition, whereas defects in DNA repair pathways, in particular mismatch repair, are well described in colorectal adenocarcinoma, no prior reports that address this issue in collision tumors are present in the PubMed database.