Most case series of Fournier's gangrene (FG) do not list malignancy as a cause; however, isolated cases with underlying malignancy of the soft tissue, genitourinary, and gastrointestinal systems have been described. After a review of recently published literature, 20 case reports and 15 case series or review articles included relevant information and were included in this literature review. Malignancy is overlooked in 10% (2/20) of patients, resulting in a delayed diagnosis and initiation of cancer treatment. All patients with FG should have a thorough cancer history, digital rectal examination, appropriate local and systemic imaging, as well as tissue biopsies, to reduce the likelihood of a missed cancer diagnosis. Delay in management of the local malignancy may lead to persistence or recurrence of the infection and significantly worsens overall outcome and survival.