Primary splenic lymphoma represents a rare entity that constitutes less than 1% of non-Hodgkin lymphomas, and less than 2% of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of primary splenic lymphomas. DLBCL encompasses a heterogeneous entity with distinct morphological variants. The anaplastic variant of DLBCL was first defined in the 2017 World Health Organization classification as a rare histological subtype that constitutes less than 3.4% of DLBCL cases.