Abstract: Objective To investigate the clinicopathological features, immunophenotype, molecular characteristics, differential diagnosis, and prognosis of follicular lymphoma with anaplastic morphology. Methods Immunohistochemical EnVision two-step method was used to detect the expressions of CD20, PAX5, CD10, BCL6, MUM-1, BCL2, CD30, Ki-67, and other markers in three cases of follicular lymphoma with anaplastic morphology. In situ fluorescence hybridization was used to detect BCL2, BCL6, and MYC gene translocations. The clinicopathological features, diagnosis, differential diagnosis, and prognosis of follicular lymphoma with anaplastic morphology were analyzed. Results The follicular lymphoma with anaplastic morphology in this study exhibited high-grade features and occurred in middle-aged and elderly patients. Immunohistochemically, the tumors expressed panB markers, were negative for CD10, positive for BCL6, mostly positive for MUM1, and positive for c-myc expression. However, no BCL6 or MYC gene translocations were detected. Conclusion Follicular lymphoma with anaplastic morphology is uncommon and mostly presents as high-grade follicular lymphoma. It is important to correctly recognize its various morphological variants.