Background The incidence of colorectal cancer in young patients is increasing. It remains unclear if the disease has unique features in this age group. Methods This was a single-center, retrospective cohort study which included patients diagnosed with colorectal cancer at age ≤40 years in 1997–2013 matched 1:2 by year of diagnosis with consecutive colorectal cancer patients diagnosed at age >50 years during the same period. Patients aged 41–50 years were not included in the study, to accentuate potential age-related differences. Clinicopathological characteristics, treatment, and outcome were compared between groups. Results The cohort included 330 patients, followed for a median time of 65.9 months (range 4.7–211). Several significant differences were noted. The younger group had a different ethnic composition. They had higher rates of family history of colorectal cancer ( p  = 0.003), hereditary colorectal cancer syndromes ( p  < 0.0001), and inflammatory bowel disease ( p  = 0.007), and a lower rate of polyps ( p  < 0.0001). They were more likely to present with stage III or IV disease ( p  = 0.001), angiolymphatic invasion, signet cell ring adenocarcinoma, and rectal tumors ( p  = 0.02). Younger patients more frequently received treatment. Young patients had a worse estimated 5-year disease-free survival rate (57.6  vs. 70 %, p  = 0.039), but this did not retain significance when analyzed by stage ( p  = 0.092). Estimated 5-year overall survival rates were 59.1 and 62.1 % in the younger and the control group, respectively ( p  = 0.565). Conclusions Colorectal cancer among young patients may constitute a distinct clinical entity. Further research is needed to validate our findings and define the optimal approach in this population.