OBJECTIVE: To evaluate the autonomic activity of patients with acrocyanosis by using heart rate variability indices. MATERIAL AND METHODS: The study group consisted of 24 patients with acrocyanosis and the control group contained 22 sex- and age-matched healthy subjects. All subjects underwent 24-hour Holter monitoring. Among the heart rate variability (HRV) parameters, time-domain and frequency-domain indices were analysed. RESULTS: The time-domain indices of HRV indicating global autonomic functions were found to be increased, and indices indicating parasympathetic activity showed a significant decrease in the patient group. Power-spectral analysis of HRV revealed that the low frequency and high frequency power were higher in the patient group than in controls. However, the ratio of Low Frequency/High Frequency was found to be lower in the patient group than in controls. CONCLUSION: In acrocyanosis, both sympathetic and parasympathetic systems seem to be disrupted. Therefore, we may conclude that acrocyanosis may be resulted of systemic autonomic imbalance rather than pure sympathetic over-activation. Also, these results suggest that acrocyanosis is not a localised disorder; on the contrary, it is associated with various abnormalities of the systemic autonomic nervous system.