BACKGROUND Multicentric reticulo-histiocytosis also known as lipoid dermoarthritis is a rare systemic disease leading to a massive osteoarticular destruction and systemic complications. METHODS This case report is a 44 year old black woman who was first seen with a rheumatoid arthritis clinical presentation associated with the presence of rheumatoïd factor. Five years later the diagnosis has been reconsidered after skin nodules histological examination. After that the patient has been lost from the follow up clinic. After a twenty years evolution she presented a complex clinical picture including: a cutaneous syndrome with a non pruriginous and hyperchromic papulonodular rash on the arms and fore-arms; a very destructive polyarthritis with major handicap; and systemic manifestations like cardiomyopathy with heart failure. The heart failure treatment associated first corticosteroids and secondary chloroquine was successful. CONCLUSIONS The rheumatoid factor presence should not avoid to consider the possibility of multicentric reticulohistiocytosis in case of polyarthritis associated with a papulonodular rash. Then skin biopsy must be performed. The severity of osteoarticular and systemic lesions require an early prescription of a treatment for which there is so far no compromise.