Patients suffering from hematological malignancies are at increased risk of Fournier’s gangrene (FG) due to immunosuppression caused by the disease itself or by disease-related treatments. A systematic review of PubMed, ISI Web of Knowledge, and Scopus databases was performed in June 2021. We included full papers that met the following criteria: original research, human studies, and describing clinical presentation, treatment, and outcomes of FG in patients with oncohematological diseases. We identified 35 papers published from 1983 to 2021 involving 44 patients (34 males, 8 females) aged between 4 days and 83 years. The most common malignant hematological disorders were acute myeloid leukemia (n = 21) and acute lymphocytic leukemia (n = 9). In 10 patients FG represented the first presentation of hematological malignancy. Scrotum (n= 27) and perineum (n = 11) were the sites most commonly involved. Pseudomonas aeruginosa (n = 21) and Escherichia coli (n = 6) were the most commonly isolated microorganisms. Surgery was performed in 39 patients. Vacuum-assisted closure and hyperbaric oxygen therapy were adopted in 4 and in 3 patients, respectively. Recovery was achieved in 30 patients. FG-related mortality was observed in 11 patients. FG should be carefully considered in patients with oncohematological diseases.