Malrotation of the gut is a congenital anomaly and usually presents in childhood. Rarely, it may present in adults. Patients may be asymptomatic, and malrotation is detected during investigations, operation or autopsy. It can cause longstanding abdominal symptoms like pain, dyspepsia or acute abdomen due to volvulus. In adults, malrotation is found with different gastrointestinal malignancies like gastric, hepatobiliary, pancreatic and, in particular, colorectal neoplasms. We are reporting a case of 60-year-old female presented with carcinoma caecum along with malrotation of the gut. It is the first case report from India. We also reviewed documented cases of malrotation associated with colorectal malignancies. A large number of cases have been reported in Japan as compared to rest of the world. Malrotation in adults is probably associated with gastrointestinal malignancies. Possible causes of this association can be genetic factors or gut changes like chronic inflammation. These associations need further study to consider intestinal malrotation as premalignant lesion which may be very important in follow-up of children with malrotation.