A study of Sudden Infant Death Syndrome (SIDS) with maternal cocaine exposure was undertaken using the perinatal medical records at the University of Miami/Jackson Memorial Medical Center and case files of the Metropolitan Dade County Medical Examiner Department, Miami, Florida USA from 1988-1992. 78 SIDS cases were analysed. 18 infants were classified as cocaine-exposed SIDS infants with positive history of maternal cocaine abuse and/or positive urine toxicology; 50 infants were classified as cocaine-negative SIDS infants without the history and with negative toxicology. The incidence of SIDS among cocaine-exposed infants was estimated to be higher than that of cocaine-negative infants overall (p < 0.025), but statistical significance between the groups was not confirmed when controlled for ethnicity. No significant differences of birth weight, estimated gestational age, body and brain weights were observed at autopsy between the two groups. Cocaine-exposed SIDS cases included 6 infants born small for gestational age (p < 0.05). 10 of 18 cocaine-exposed SIDS infants had a positive result for urine toxicology at birth and died younger aged than cocaine-negative infants (p < 0.05). Toxicology tests of the specimens obtained from cadavers were all negative for cocaine metabolites.