Abstract: Approximately 8% to 15% of patients with metastatic colorectal cancer (mCRC) harbor BRAF mutation, and the V600E mutation is the most common form of BRAF mutation. The prognosis of patients with metastatic colorectal cancer harboring BRAF V600E mutation is poor. Initial standard chemotherapy is often ineffective, necessitating an intensive follow-up treatment, which usually provides limited efficacy. Consequently, the disease becomes notably difficult to treat and progresses rapidly, resulting in a decreased overall patient survival rate. This review details the research advancements in treatment of BRAF V600E-mutant metastatic colorectal cancer.