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Background: T‐cell‐rich B‐cell lymphoma (TCRBCL) is an uncommon B‐cell lymphoma, which is characterized histologically by a small number of neoplastic B cells surrounded by large numbers of nonneoplastic T cells. Diagnosis is frequently difficult because the neoplastic B‐cell population may be quite sparse, and immunohistochemical and molecular analysis to identify the B‐cell origin and clonality of the cells is necessary. Methods: In this report we present an additional case of primary cutaneous TCRBCL with immunohistochemical and gene rearrangement studies and review the literature of this unusual entity. Results: The patient was a 52‐year‐old woman who had a slowly growing cutaneous lymphoma which fulfills the strictest criteria initially proposed for a diagnosis of TCRBCL. This case has additional features of a prominent angiocentric/angioinvasive histology and dual clonality, showing both immunoglobulin heavy chain and T‐cell receptor gene rearrangements. Conclusions: Controversy surrounds the notion of TCRBCL as a distinct clinicopathological entity. There have only been 14 reported cases of TCRBCL with skin involvement in the literature and only seven of these cases have arisen in the skin. To better understand the histological and clinical characterisitcs of TCRBCL we have reviewed previously reported cases of TCRBCL with skin involvement, and contrast them with the present case.

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