Familial hypercholesterolemia (FH) is an autosomal dominant genetic disorder and characterized by hypercholesterolemia and early onset atherosclerosis. It may be misdiagnosed as rheumatoid arthritis (RA) because of swelling in joints and periarticular area can mimic arthritis or rheumatoid nodules. This report presents the case of a patient with FH manifested by seropositive RA. Treatment included lipid lowering therapy and management of RA. It is important to recognize the association between rheumatologic manifestations and familial hypercholesterolemia for diagnostic and therapeutic reasons.