As yet, there have been neither systematic reviews nor reports of randomized, controlled trials involving factor VII (FVII) deficiency. Hence, a picture of this disorder can only be drawn by reviewing and summarizing the data that is available. This article provides an overview of the understanding of this rare, inherited disorder of coagulation. In particular, the status of current knowledge of the disorder's prevalence, clinical presentation, diagnostic characteristics and molecular genetics is reviewed, followed by a summary of currently available options for its treatment and management.