Chondromyxoid fibroma of the skull base is extremely uncommon. Sometimes involvement of the nasal cavity may occur and the patients may present with nasal symptoms. The biological behaviour of this tumour has not been well studied, primarily because of the limited number of reported cases and the short duration of follow-up. We report a histologically confirmed case of chondromyxoid fibroma of the skull base that recurred repeatedly over a 10-year period after the initial operation. Histologically it showed identical morphology to the original tumour with no evidence of histological progression or dedifferentiation. Ultrastructurally, the spindle tumour cells in the fibromyxoid area showed dual chondroblastic and fibroblastic differentiation, suggesting that these spindle fibroblastic cells and the better differentiated chondroid cells were of the same cell type with different histological morphology.