Reduced heart rate variability has been found in infants who later succumb to the sudden infant death syndrome (SIDS). To determine whether respiratory sinus arrhythmia, a major component of heart rate variability, is also reduced in SIDS victims, nighttime portions of eighteen 24-h recordings of ECG and respiration from infants who later died of SIDS and 52 recordings from control infants were assessed using spectral analysis. Two aspects of respiratory sinus arrhythmia were examined: “extent” (the absolute heart rate variation at the respiratory frequency) and “coherence” (the degree to which heart rate follows respiration regardless of the absolute amount of variation). Respiratory parameters were used to classify each 1-min epoch as quiet sleep, rapid eye movement sleep, waking, or indeterminate state. Median extent and coherence values across the night were then computed for each sleep-waking state. Two-way (group ± state) repeated measures analysis of variance tests were then used to compare respiratory sinus arrhythmia values for 13 SIDS victims and 13 control infants matched by postnatal age, birth weight, sex, and gestational age. Extent of respiratory sinus arrhythmia was significantly lower in the SIDS victims across all sleep-waking states, a finding that persisted after adjusting for heart rate. Coherence values did not differ significantly. These results suggest that even before the time of maximal risk for the syndrome, SIDS victims, as a group, differ from controls in the extent to which cardiac and respiratory activity couple, and this difference is independent of basal heart rate.