A retrospective review of 47 cases of lymphomatoid granulomatosis encountered between 1977 and 1990 revealed that 24 patients had skin involvement. Clinical and histologic features were noted. The dominant and most characteristic cutaneous finding was scattered nodules. Other cutaneous changes included eroded and crusted lesions, facial edema, papules, and folliculitis-like eruptions. The nodular skin lesions most often had an angiocentric and angiodestructive dense, polymorphous, lymphohistiocytic infiltrate in the dermis and fat. A minority of the cases had a lymphoma cutis type of infiltrate. Immunophenotypic analysis showed a mixed population of T cells, with helper T cells (CD4+) predominant. Our results emphasize the usefulness of the clinical and histologic examination of the skin in lymphomatoid granulomatosis. The histologic pattern of lymphomatoid granulomatosis was seen in the skin in more than half of the biopsy specimens.