Sudden cardiac death (SCD) is the most common natural cause of death. The hypothesized mechanism of death is an arrhythmia precipitated by increased sympathetic outflow. The left stellate ganglion provides sympathetic innervation to the heart and plays a role in arrhythmogensis. We present a SCD with stellate ganglionitis in which the inflammatory cells were characterized. The case was 37-year-old man who died from ischemic and hypertensive heart disease. The left stellate ganglion showed lymphocytic inflammation with features of humoral immune response. This case report provides evidence that stellate ganglionitis can be seen in SCD and raises the possible association between the two. •Sympathetic outflow can trigger sudden cardiac death (SCD).•The heart receives sympathetic innervation from the stellate ganglion.•Stellate ganglionitis (SG) is associated with cardiac arrhythmia and SCD.•SG in SCD is poorly described and not well recognized.•We report a SCD showing SG with evidence of humoral immune response.