Schistosoma japonicum is a digenetic blood fluke that has been implicated in the carcinogenesis of several human malignancies, notably liver and colorectal cancer (CRC). Schistosoma japonicum-associated colorectal cancer (SACC) is a distinct subtype with biological behavior analogous to colitis-induced CRC. The clinicopathological characteristics of SACC include young age at diagnosis, predominance among males, a strong predilection for the sigmoid colon and rectum, multifocal distribution, frequent mucinous histology, and poor prognosis. In addition to chronic inflammation, immunomodulation, and schistosomal toxins, bacterial coinfection appears to play an important role in the carcinogenic process. The present review provides the most recent updates on epidemiology, pathobiology, and clinical and prognostic features pertaining to SACC.