We report detailed clinicopathologic features of 3 cases of plasmablastic lymphoma (PBL) with MYC/IgH rearrangement, representing one third of PBL cases diagnosed at our institution. This study brings the total number of reported cases in the literature to 6. All patients were HIV+ with very low CD4 counts at diagnosis. The involved locations were mediastinum, anus, and bone marrow. Tumors exhibited predominantly immunoblastic/plasmablastic morphologic features and had a plasma cell-like immunophenotype. Bright CD38 expression by flow cytometry had a tendency to be more common in these cases compared with PBL without MYC rearrangement. All cases were positive for Epstein-Barr virus-encoded RNA but lacked human herpesvirus-8 latent nuclear antigen. The 2 patients with follow-up died within 3 months. These findings show that PBL is often associated with MYC/IgH rearrangements and that this finding may portend an aggressive clinical course, suggesting that cytogenetic studies should be routinely applied in cases of PBL.