The aim of this study is to report the relative frequencies of non-Hodgkin lymphoma (NHL) subtypes in Guatemala. A panel of five hematopathologists reviewed 226 consecutive biopsies and classified them according to the 2001 World Health Organization (WHO) classification. The 83 cases of diffuse large B-cell lymphoma (DLBCL) were further subclassified into germinal center B-cell-like (GCB) and non-GCB subtypes. Of the 226 cases, 194 (86%) were confirmed as NHL, including 169 (87%) B-cell and 25 (13%) T- or natural killer (NK)-cell NHL. The most common subtype was DLBCL (44.3%), and the most frequent subtype among T- and NK-cell NHL was extranodal NK/T-cell lymphoma, nasal type (7.8% of all NHL). A comparison of the frequencies of NHL subtypes between Guatemala and other parts of the world showed that Guatemala is most similar to the Middle East and Asia. However, there is no significant difference in the frequency of the DLBCL subtypes compared to North America and Europe.