Neurofibromatosis type 1 (NF1) is a disease characterized by increased tumorigenesis susceptibility, caused by mutations of the oncosuppressor gene NF1. The glomus tumor (GT) is a rare, very painful mesenchymal neoplasm, arising from the glomus body. In recent years, it has been highlighted the association between NF1 and GT. We report a case of a man aged 65 years, suffering from NF1, with intense pain at the thenar eminence of the right hand, successfully treated with the excision of the mass.