Goodpasture syndrome is a rare immunologically based clinical entity that is characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage, and the presence of circulating autoantibodies against the glomerular basement membrane (GBM). Concomitant central nervous system (CNS) manifestations are an unusual presentation of GSP in the absence of ANCA. We present a case of atypical course of the disease in a 48-year-old female patient who started with the presence of hemoptoic sputums and manifestations of uremic syndrome in the presence of high titres of glomerular basement anti membrane antibodies (Ac-anti-MBG) and negative ANCA, with a report of diffuse active extracapillary proliferative glomerulonephritis biopsy, of the pauciimmune type. After immunosuppressive treatment and plasmapheresis, the patient presented decreased anti-MBG Ab titres, as well as improvement of symptoms, however without recovery of renal function on hemodialysis, after 2 months he presents clonic-tonic seizures crisis, we conclude that PGS with neurological involvement is extremely rare, especially with negative ANCAs.