Mucormycosis is an emerging infection with a predilection for patients with impaired phagocytic immune function caused by either an underlying disease or its treatment. Although a rising incidence in hematology wards has generated increased interest, the current appreciation of gastrointestinal (GI) mucormycosis is limited. Mortality associated with GI infection remains high, due in part to poor disease recognition by clinicians and subsequent delays in diagnosis and the provision of appropriate antifungal therapy.Through a review of the English language literature, we were able to identify 22 cases of primary GI mucormycosis in patients with hematologic malignancies, in addition to a 23rd case which we report here. The most common presenting symptoms were nonspecific and included fever (70%), abdominal pain (65%), and abdominal distention or ileus (48%), with neutropenia documented in 74% of individuals at the time of symptom onset. Necrotizing enterocolitis was the most common manifestation of GI infection, seen in 78% of individuals, with the ileum and cecum the most frequently affected sites.To our knowledge, this review is the first to focus on the most common signs, symptoms, and image findings in patients with hematologic malignancy developing primary GI mucormycosis. Our findings call into question several widely accepted beliefs within the medical literature, namely that the stomach is the most common site of infection and necrotizing enterocolitis is an unusual presentation in adults.