Fifty cases of malignant lymphoma, exclusive of mycosis fungoides, with initial involvement of the skin are reported. The head and neck was the most common site of presentation and 44% of the cases were classified as the diffuse histiocytic type. The cases encompassed virtually the entire spectrum of histologic subtypes of non-Hodgkin's lymphomas; these subtypes were a major prognostic factor in predicting the subsequent clinical course. Among 37 patients with available follow-up information, those patients with a "favorable" histology had a significantly better five-year freedom from relapse (70% vs. 19%) and survival (88% vs. 30%) compared with those patients with the "unfavorable" types of lymphoma. clinical staging was another important variable in determining prognosis. Fifteen patients with disease confined to skin had a significantly longer freedom from relapse (44% vs. 23%) at five years than 22 patients in whom staging procedures revealed extracutaneous disseminated lymphoma. Thirteen of the patients with widespread lymphoma have died from 2-30 months after diagnosis (median survival ten months), in contrast with only four deaths among the patients with limited disease. The pattern of relapse for these latter patients tended to involve cutaneous sites distant to the original lesion. This observation may have a bearing on future therapy of cutaneous lymphomas.