Primary tumours of the first rib remains a rarity, but the diagnosis and the surgical approach are quite challenging because of the anatomical difficulties in approaching it and the closeness of subclavian vessels. The anterior transmanubrial approach gives an excellent exposure, especially when approaching the anterior part of the first rib. Diagnosis quite often can be approved just by the histo-pathological examination of the resected specimen. We describe the management of such a case, when diagnosis of fibrous dysplasia was confirmed postoperatively.