Heterozygotes or homozygotes with familial hypercholesterolemia may have recurrent attacks of polyarthritis and tenosynovitis, especially in the ankles, knees, wrists, and proximal interphalangeal joints. In one study 40 percent of adult heterozygotes had at least one episode of articular pain. Achilles pain or tendonitis occurred in 29 percent, oligoarticular arthritis in 7 percent, and polyarticular or rheumatic fever like arthritis in 4 percent. Fever, leukocytotis and elevated sedimentation rate occur infrequently. Antiinflammatory drugs do not seem to influence the course of these attacks. The elevation of ESR is presumably related to the high plasma LDL levels. In this study a rheumatic fever like case with hypercholesterolemia was discussed and presented clinical and laboratory results.