Scleromyxedema is a rare fibromucinous disorder also known as Arndt–Gottron disease. The diagnosis must fulfil the following criteria: generalised papular and sclerodermoid eruption, monoclonal gammopathy (mostly IgG, light lambda chains), no signs of thyroid disorders (differentiation with pretibial myxedema), and pathognomonic biopsy result (triad: mucin deposition, fibroblasts proliferation, fibrosis). In 60–90% of patients with scleromyxedema organs other than the skin are affected, and in 10–15% of patients the central nervous system may be involved. The aetiology of the disease still remains unclear. Currently, IVIg is considered the best therapeutic option. We present the case of a 64-year-old man with a 14-year history of scleromyxedema currently treated with intravenous immunoglobulins, in whom we observed episodes of transient aphasia, and aphasia with hemiparesis, with no radiological evidence of acute cerebral lesions. The patient was treated with rt-PA with initial good results. Dermato-neuro syndrome was considered as a cause of the patient’s symptoms