Abstract: Cronkhite-Canada syndrome (CCS) is a rare cause of chronic diarrhea and malabsorption where patients develop multiple polyps throughout the gastrointestinal (GI) tract, accompanied by ectodermal changes. Due to its rarity, its early detection and diagnosis can be challenging for physicians. This case report described a 58-year-old male patient with CCS who presented with chronic watery diarrhea, hematochezia, weight loss, and skin changes including nail dystrophy and hyperpigmen-tation. Laboratory results showed anemia and hypoalbuminemia. He underwent CT enterography (CTE) which identified diffuse edematous polyposis in the GI tract. The CTE results were highly suspicious of CCS and a subsequent endoscopic examination confirmed the diagnosis. The patient received supportive treatment which improved his symptoms. Based on CTE and endoscopy at 1-year follow-up, the patient was deemed to be in remission. We included a literature review of CCS. The case report aimed to improve the understanding of CCS and explored the key CTE features relevant to its early diagnosis.