Colorectal cancer (CRC) is one of the 3 most common types of cancer in women, but CRC during pregnancy is rare, with a reported incidence of approximately 0.002%. Synchronous colon cancer during pregnancy presents a diagnostic and therapeutic challenge for clinicians because there are no generally accepted guidelines regarding diagnosis or treatment. The diagnosis is challenging because the presenting signs/symptoms of CRC are often attributed to the usual complications of pregnancy, which could delay the diagnosis and allow the cancer to progress to an advanced stage. Carcinogenesis of colon cancer in pregnancy is not clear, but a few studies suggest that the increased levels of estrogen and progesterone related to pregnancy stimulate the growth of CRC with their receptors. The aim of treatment is to start therapy for the mother as early as possible and to simultaneously deliver the baby at the earliest time allowable. The management mandates a multidisciplinary approach involving experts in obstetrics, neonatology, gastrointestinal surgery, and medical oncology. The medical community should be able to diagnose colon cancer earlier in pregnancy in order to improve prognosis. The primary care physician or obstetrician should refer the pregnant patient with significant gastrointestinal symptoms to the gastroenterologist for evaluation. Likewise, the gastroenterologist should be prepared to perform sigmoidoscopy (preferably without endoscopic medications) for significant lower gastrointestinal symptoms such as persistent rectal bleeding. Herein, the author reviews the literature concerning the diagnosis and treatment of CRC in pregnancy and discusses the role of newer agents approved for the treatment of CRC.