Background Implementation of screening modalities has led to a decreased incidence of colorectal malignancies. Unfortunately, overall incidence has remained unchanged as cases have increased in patients below the suggested screening age. Therefore, we evaluated characteristics and oncological outcomes of malignancies in patients ≤40 years of age. Methods Single-center retrospective analysis of prospectively collected data of malignancies in patients ≤40 years evaluated in our institution between 2010 and 2016. Basic descriptors for demographic, clinical, histologic, and genetic data were collected. Disease-free survival (DFS) and 5-year overall survival (OS) were compared for patients between 30-40 years and <30 years. Results Fifty-six patients ≤40 years were identified, 44 of whom (96.5%) had adenocarcinomas. Most common malignancy location was the rectum (64.3%). Despite aggressive tumor characteristics such as moderate/poor differentiation (88.6%), lymphovascular invasion (26.8%), perineural invasion (21.4%), and advanced tumor stage T3/T4 (60.7%), OS rate was 94.6%. Both age groups had similar oncologic characteristics. There was a trend toward worse OS (2/11 and 1/45, P = .06) but not for DFS (7/11 and 15/43, P = .18) in patients <30 years of age compared to 30-40 years. There were no differences in OS (3/44 vs 0/88, P = .44) or DFS (17/42 vs 3/8, P = .80) between sporadic vs non-sporadic malignancies, respectively. Conclusions Patients ≤40 years of age with malignancy have advanced tumor stages and aggressive tumor characteristics at diagnosis. Although there is higher OS risk for patients <30 compared to those aged 30-40 years, no differences were found for DFS between these two groups.