Two cases of malignant lymphoma in childhood were studied. The first case was a Japanese girl aged 8, in whom the primary site was skin of the right temple. The second case was a 4-year-old Japanese boy, who had metastases to the abdominal skin. Histochemical findings indicated B-cell lineage in both cases. Primary cutaneous lymphoma is extremely rare in childhood. Fourteen such cases that have been reported in Japan and our case added to them, were reviewed. The relationship between their morphologic, immunohistochemical and clinical findings were summarized and discussed. Although the prognosis of lymphoma confined to skin in childhood has been reported not to be bad as compared with other types of lymphoma, our first such case was fatal. This suggests that appropriate initial treatment is very important. Recent advances in science may clarify the clinical and biologic characteristics of this tumor in the near future.