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Background: Primary colorectal signet ring cell carcinoma (SRCC) is rare and often affects younger patients. It demonstrates a different biological behaviour and has a less favourable prognosis than the more conventional colorectal adenocarcinoma., Case Description: Patient A, a 65-year-old man, presented with a change in bowel habit. Colonoscopy showed a stenosis with signs of a nonspecific inflammation. However, laparoscopy revealed a primary colorectal SRCC with diffuse peritoneal metastases. Standard chemotherapy was unsuccessful. Patient A died 13 months after diagnosis., Conclusion: Based on clinical and molecular differences, SRCC can be recognized as a separate subtype of colorectal cancer. The case description illustrates that this group of patients may benefit from a different management strategy than the usual therapy. A greater understanding of the characteristics of this specific malignancy is needed.