Two cases of colorectal mucosa-associated lymphoid tissue lymphoma (cMALT) are presented and discussed with the reports from 1997 to the present. Helicobacter pylori (HP)-negative cases showed tumor resolution 2 months after eradication therapy. HP-positive cases were successfully eradicated and tumor resolution was confirmed 16 months later. Analysis of the data reported to date shows that cMALT resolution rates were 68.4% (13/19) in the HP-negative group and 33.3% (7/21) in the HP-positive group. HP eradication should be considered the primary treatment for cMALT regardless of HP infection, especially in untreated patients under follow-up.