Abstract: With the development of precision medicine, targeted therapy has achieved good efficacy in patients with partial advanced non-small cell lung cancer (NSCLC) with a positive driver gene. Anaplastic lymphoma kinase (ALK) is called the diamond mutation due to its low mutation rate and high survival rate. In the general population, the incidence of arrhythmia caused by ALK inhibitors (ALKIs) is much higher than that reported in clinical trials. Severe arrhythmia can lead to drug reduction, drug withdrawal, pacemaker installation, and even sudden death. This review focuses on the research progress of ALKIs related arrhythmia with the aim of improving clinicians’ understanding of the adverse effect and therefore guide the usage of medication.