We describe a 62-year-old woman who presented with a recurrent neurofibroma, two ‘café-au-lait’ spots and a possible cerebral tumor. This Von Recklinghausen neurofibromatosis was associated with an asymptomatic systemic mastocytosis with bone marrow involvement. We discuss Riccardi’s recent theory on the pathogenesis of neurofibroma which postulates that the mast cells have a primordial role in the development of neurofibroma. Our observation could be additional evidence in favor of this