A malignant rhabdoid tumor was first described as a subtype of Wilms tumor in 1978. The most frequent location of these tumors is the kidney, and they are common in childhood. The extrarenal localization of these tumors has been described mainly in the central nervous system (called atypical teratoid-rhabdoid tumors), liver, soft tissues and colon. Localization in the small intestine is uncommon and since the 1990s, only a few cases of malignant rhabdoid tumors in the small intestine have been reported. This tumor is very aggressive and the prognosis is poor. We herein present our personal experience with a rhabdoid tumor of the jejunum in a 76-year-old male, and also provide an analysis of the cases of malignant rhabdoid tumor of the small intestine previously described in the literature as for a brief review. We also compared the previous reports and our present case to try to identify prognostic factors.