The presence of blood in the semen--hematospermia--is an anxiety-provoking but usually self-limiting condition. Its exact incidence remains unclear. The risk of underlying malignancy, despite being very low, drives evaluation. There is confusion amongst clinicians as to how best to refer, evaluate and treat men with hematospermia. We have reviewed the literature in an attempt to present a cohesive view of the etiologies and diagnostic and management strategies in patients with this condition.