This is a ten years retrospective study conducted to assess the clinical profile and outcome of Guillain-Barré Syndrome (GBS) in Ethiopian patients. The medical records of all patients admitted with the diagnosis of GBS to the Department of Internal Medicine, Tikur Anbessa University Hospital, Addis Ababa, Ethiopia during the period September 1992 to September 2001 were reviewed. During the ten-year review period ninety-five patients were admitted with the diagnosis of GBS, of which eighty-one met the National Institute of Neurological and Communicative Disorders and Stroke diagnostic criteria. The mean age (SD) of patients was 34.4 +/- 14.4 years. The male to female ratio was 1.25:1. History of antecedent event such as upper respiratory tract infection, diarrhea and vaccination was obtained in 47/81 (58.1%) of patients. The majority of the patients 62 (78.5%), presented with an ascending areflexic quadriparesis while 10 (12.7%) presented with a descending type of arefilexic quadriparesis, but in 6 (7.4%) of the patients the weakness was confined to the lower extremities (i.e. Paraparetic variant). Signs of autonomic involvement were seen in 25/81 (30.9%). Cytoalbzuminological dissociation in the cerebrospinal fluid was demonstrated in 62.3%. EMG was done in 47 patients The commonest electrophysiologic abnormality encountered was demyelinating picture in 26 (55.3%) followed by mixed and axonal in 12 (25.5%) and 9 (19.1%) respectively). Nineteen (70.3% of 27 GBS patients for whom serology for HIV was done were seropositive. The clinical findings were similar in both groups, except for a higher frequency of CSF pleocytosis, need for ventilatory support and mortality among HIV-positive patients. The overall mortality was 25.9%. This study highlights the importance of GBS as a cause of peripheral nerve disease in our setup. The higher mortality rate observed in this study appeared to be related mainly to the lack of adequate intensive carefacility and highlights the need to improve these services. Further-more, the finding of high frequency of seropositivity among GBS patients in this study underlines the need for further prospective research on the association and clinical course of GBS and HIV, particularly in Africa where HIV/AIDS has become an important public health problem.