Intestinal malrotation is an infrequent congenital anomaly. Its presentation in adults is rare, and it is usually discovered incidentally. This article presents an extremely rare case of an adult patient presenting with obstructing colon cancer associated with intestinal malrotation. This is the ninth case to be published in the past 40 years. After proper resuscitation and imaging, an open resection was performed for the patient due to unresolving obstruction and significant abdominal distention. This case highlights the rarity of colon cancer in a malrotated gut and the importance of preoperative evaluation of the unique anatomy before surgical intervention. It also discusses the possible surgical options for such patients with obstruction due to colon cancer causing suboptimal circumstances for both minimally invasive intervention and reestablishment of bowel continuity.