Primary colorectal lymphoma is rare, representing less than 1% of colorectal malignancies. Males, in the 5th–7th decades of life, are most commonly affected. The most common location is the cecum. The most common histology is B-cell lymphoma, which can be rapid or indolent in presentation. Aggressive forms are more likely to require immediate intervention due to symptoms, whereas indolent non-Hodgkin lymphoma typically progresses slowly over months to years. Lymphoma is highly treatable with systemic therapy. Management decisions are made in consultation with a hematologist-oncologist.