Mantle cell lymphoma (MCL) is a clinicopathologic entity with distinctive morphologic and immunophenotypic features and a characteristic cytogenetic abnormality, the t(11;14)(q13;q32). Although MCL was recognized over 30 years ago, a lack of consensus regarding its morphologic features precluded its inclusion into non-Hodgkin's lymphoma (NHL) classification schemes until relatively recently. An accurate diagnosis of MCL is of great importance, since this tumor generally carries a poor prognosis and requires more aggressive and novel treatment regimens. In this article, we briefly overview the clinical features of MCL and then focus on the pathologic diagnosis of MCL, emphasizing morphologic findings and various ancillary techniques useful in the diagnostic workup. Involvement of lymph nodes and other sites, such as the spleen, liver, gastrointestinal tract, Waldeyer's ring, bone marrow, peripheral blood, and cerebrospinal fluid are reviewed. The diagnosis of high-grade variants of MCL is a particular challenge, as these tumors exhibit a broad spectrum of morphologic findings that can be misinterpreted as other types of NHL. The molecular basis of MCL is also briefly reviewed to highlight the biologic role of the t(11;14) and cyclin D1 overexpression in this tumor and the value of immunophenotypic and molecular methods for their detection as diagnostic aids.