Chronic recurrent multifocal osteomyelitis (CRMO) and B-cell lymphoma are fairly uncommon conditions that are seen in children. Although CRMO can leave patients with residual deformities and disabilities, it is a benign condition. We describe an unusual situation in which a site adjacent to a biopsy-documented site of CRMO presented 3.5 years later with B-cell lymphoma. B-cell lymphoma can behave in an indolent manner and reports suggest that this condition can initially be mistaken for CRMO. This case presented here underscores the importance of vigilance in the short and long-term management of patients initially diagnosed with CRMO, and raises the possibility that the pathogenesis of the two conditions is related.