Histologic transformation in the course of non-Hodgkin's lymphoma (NHL) has been reported to occur in 18 to 30% of the cases. Less favorable prognosis in cases with initial low grade malignancy followed by emergence of high grade malignancy has been previously described. In the previous literature, the histologic transformation has been examined mostly in nodal NHL. In the present study, histologic transformation in the course was investigated on 20 cases with early extranodal NHL. All these 20 cases were diffuse lymphomas, and were composed of 7 cases with low grade malignancy and 13 cases with high grade malignancy. Histologic transformation was not observed in any of these cases. These findings indicate that the frequency of histologic transformation is much lower in extranodal NHL than in nodal NHL. The prognostic significance of these findings is also discussed.