Abstract: Neonatal lupus erythematosus (NLE) is a rare disorder believed to be caused by the transplacental passage of specific autoantibodies from the mother to the fetus. Affected infants typically present with either characteristic skin lesions developing in the first month or later in life or congenital heart block. We report a very unusual case of NLE that was characterized by congenital skin atrophy, erosions, alopecia, and profound pancytopenia. Ro, La, and RNP antibodies were absent, and the diagnosis was made because of light microscopic and immunofluorescent findings. This case emphasizes that NLE can have widespread congenital skin involvement and suggests that at least some cases are mediated by antibodies other than Ro, La, and RNP.