Penile cancer is a rare malignancy which HIV infection appears to increase the risk of. The magnitude of this risk and the pathogenesis remain unclear. A comprehensive review of the literature was undertaken using conventional search strategies. Twenty-four publications were identified by this methodology, of which nine were case reports and 15 were observational studies. These studies were highly heterogeneous, with varying study designs, populations, and objectives. The risk of penile cancer within HIV-positive individuals is significantly greater than in those without HIV (RR = 3 .7 to 5.8, 3 studies; SIR = 3.8 to 11.1, 4 studies). HIV is also shown to influence disease characteristics, with a four-fold increased risk of death from penile cancer. Moreover, progression from intraepithelial neoplasia occurs earlier in HIV, six years sooner than in HIV-negative men. HIV-positive men have a higher prevalence of HPV infection. Ethnicity is also shown to modulate the relationship between HIV and penile carcinoma, with a higher risk of cancer in Hispanic, compared with Caucasian, HIV-positive men. This review has collated data from diverse sources to improve understanding of the relationship between HIV and penile cancer. This relationship has been quantitatively and qualitatively characterised and highlights areas deserving further enquiry.