Primary bone lymphoma is a rare condition which represents a low percentage of both the malignant primary bone tumours and the non-Hodgkin extranodal lymphoma. This explains the lack of publications, lines of investigations, and specific diagnostic and treatment protocols. In the following article we will carry out a revision of the existing literature on this rare subject, using as argument a clinical case of left femoral location stage IE treated with CHOP chemotherapy and radiotherapy.