We describe a 33-year-old woman with a 2-year history of rheumatoid arthritis-like joint changes and a 1-year history of papulonodules on the fingers, ears, oral mucosa, forearm, elbows and thighs, and erythematous plaques on the buttocks. Six months after the onset of the cutaneous lesions she had dyspnoea, 3 months later she developed a husky sound. Biopsies from the nodules on the ear and left forearm revealed multinucleated giant cells with eosinophilic ‘ground-glass’ cytoplasm. Bronchoscopy revealed that there were nodules in the bronchus. A biopsy taken from a nodule from the bronchus was consistent with multicentric reticulohistiocytosis. Fibrostic laryngoscopy showed a mass 1.5 × 2.0 cm2 in size on the left aryepiglottic fold and posterior commissure. Magnetic resonance imaging revealed a large pleural effusion and pericardiac effusion. Ultrasound revealed splenomegaly and peritoneal fluid. Combination therapy with prednisone, cyclophosphamide and methotrexate significantly improved cutaneous and joint symptoms. The huge cutaneous erythematous plaques and the generalized systemic involvement make this case interesting.