Head and neck follicular lymphoma (FL) with marginal zone (MZ) differentiation is a rare high-risk B-cell composite variant that has been reported in nodular but not extranodular sites in the parotid glands. Here we summarize the literature on FL with MZ differentiation in head and neck nodular sites and describe a rare case of extranodular FL with MZ differentiation in the parotid gland. We examined both the germinal center and MZ components of the parotid and bone-marrow biopsies of a 65-year-old female histologically, immunohistochemically, and molecularly to identify B-cell, germinal center, and follicular dendritic cell markers. The immunohistochemical and molecular analysis provided evidence that the FL and the MZ components derived from the same B-cell clone with a similar BCL2/IGH t(14;18) translocation site. The differentiated cells in the MZ did not express germinal center markers BCL6 and CD10. Both the parotid and bone-marrow proliferative B cells showed BCL6, CD2O, and CD79a positivity. Head and neck FL with MZ differentiation can develop in both nodular and extranodular sites and is characterized by BCL2 translocation t(14;18). Although the mechanism of MZ differentiation is unclear, the characterization of this rare histopathologic phenomenon might be clinically important.