Objective: To investigate the linkage between dentinogenesis imperfecta type II and chromosome 4q21 in a Tianjin-Tanggu family of the Hui nationality. Material and Methods: Blood samples were collected from 13 family members. DNA was analyzed with 8 STRP markers (D4S2915, D4S2932, GATA62A11, D4S2409, DSP STRP, SPP1 STRP, D4S1563, D4S1544) using fluorescence-based PCR. The linkage between each of 8 markers on chromosome 4q21 and the dentinogenesis imperfecta type II locus was tested respectively by lod-score analysis. Results: Genotypes and haplotypes were acquired. Genetic linkage analysis demonstrated that the maximum lod scores of all 8 STRPs were more than zero; 5 of them were more than 1. Conclusion: The locus of dentinogenesis imperfecta type II in this Chinese family is located on human chromosome 4q21, which indicated that the locus for the Chinese Hui nationality was the same as for other reported European or American families.