Crimean-Congo haemorrhagic fever (CCHF), a tick-borne viral zoonosis, is focally endemic throughout parts of Eurasia, the Middle East and Africa. In sub-saharan West Africa only 2 previous cases, both non-fatal, have been reported. We documented a fatal human case of CCHF in south-western Mauritania during May 1988 by demonstrating CCHF virus-specific class M antibodies and by isolating CCHF virus. Five of 7 other patients simultaneously in hospital with haemorrhagic fever symptoms also exhibited elevated immunoglobulin (Ig) G. Healthy family members and contacts of these patients showed an IgG prevalence of 36%; similarly 29% of their sheep also had antibodies. A serosurvey of 1219 sheep from 14 widely dispersed sites throughout southern Mauritania demonstrated IgG prevalences ranging from 4.9% to 43.6%. IgM was found in many herds. These observations demonstrate that CCHF virus is enzootic in southern Mauritania, and suggest a recent period of intense transmission in parts of the region.