Colorectal hepatoid adenocarcinoma is rarely reported and is known to have a poor prognosis. Reports of chemotherapy against colorectal hepatoid adenocarcinoma are scarce. Here, we provide the first report of a case involving XELOX ? bevacizumab treatment of rectal hepatoid adenocarcinoma. The patient, a 40-year-old female, was diagnosed with early rectal cancer and underwent endoscopic mucosal resection. Fourteen months later, multiple lymph node metastases appeared. She received XELOX ? bevacizumab, and maintained stable disease for approximately ten months. However, unfortunately, this regimen had to be stopped because of interstitial pneumonitis. She underwent other chemotherapies and chemoradiotherapies but died approximately two years after the recurrence. Our results indicate that the XEL-OX ? bevacizumab regimen may be effective for controlling this disease.