Background: Orofacial anomalies occur due to incomplete fusion of developmental lines in the head and neck region. Dental anomalies regarded as the most common orofacial anomalies either in isolated or syndromic forms arise due to genetic and environmental factors. Among genetic influences, consanguineous marriages are considered as a significant predisposition factor in the transmission of congenital defects and several autosomal recessive diseases from one generation to other with an increased risk of detrimental effects on offspring. Aim: The present study was aimed to evaluate the prevalence and significant association between consanguinity and isolated dental anomalies with that of nonconsanguineous parents among south-Indian population. Methodology: A total of 116 participants with and without dental anomalies in isolated form pertaining to tooth size, shape, altered morphology, number and eruption were selected followed by brief case history. Participants with a positive history of consanguinity were categorized as Group A while others were categorized under Group B. Results: Sixty-four out of 116 participants (55.17%) showed positive consanguinity (Group A) among which 18 females (56%) and 14 males (44%) presented with isolated dental anomalies. 12 females (66.6%) and 9 males (64.2%) in Group A showed significance with first cousin ( P = 0.00204) whereas no significance was observed in other consanguinity type ( P = 0.7287). Nonetheless, the overall frequency of isolated dental anomalies was slightly higher in Group A than Group B that was statistically significant ( P = 0.0213). Conclusion: A positive correlation between dental anomalies among offspring of consanguineous marriages revealed such prevalence may be attributed to increased risk of recessive deleterious gene expression or defective allele carried to offspring.