Goodpasture syndrome, a rare human autoimmune disorder, is characterized by the presence of pathogenic autoantibodies that react with the components of the glomerular basement membrane. The clinical condition of the Goodpasture syndrome is characterized by an acute necrotizing glomerulonephritis, often with accompanying pulmonary hemorrhage. Notably, the Goodpasture antigen has been localized to the noncollagenous domain of the alpha3 chain of type IV collagen. Additionally, human leukocyte antigen-DR2, and to a lesser extent human leukocyte antigen-DR4, have been identified as important restriction elements. The role of T cells in Goodpasture syndrome is indicated by the highly restricted specificity of the antibody response and the strong major histocompatibility complex class II association. In this review article, we briefly describe the latest views on the molecular and cellular themes of Goodpasture syndrome.