Primary pulmonary melanoma is a very rare disease, with only 19 cases previously reported in the English language literature. These cases suggest that melanoma can arise in the lung as a primary tumor, probably from residual melanoblasts. Primary pulmonary melanoma is frequently endobronchial and often manifests with symptoms of cough, hemoptysis, and lobar collapse. Aggressive surgical resection, irrespective of lymph node involvement, offers possible long-term survival in some patients. The diagnosis of primary pulmonary melanoma necessitates that both clinical and histologic criteria be fulfilled. Herein diagnostic criteria are proposed, and the diagnostic approach is discussed.