Background. HLA-DR1 is associated with the development of multiple basal cell carcinomas (BCC). However, the association is weak. Objective. The purpose of our study was to determine whether HLA-DR1 is a marker for susceptibility to the development of many BCCs during a lifetime. Methods. Persons with multiple BCCs were placed into two groups: those with less than 10 and those with 20 or more. In addition, the HLA-DR1 frequencies were analyzed. Results. HLA-DR1 was associated with multiple BCCs in the group with less than 10 BCCs but not with the other group. These patients were significantly younger on average than those with 20 or more BCCs. Conclusion. HLA-DRI is associated with the development of multiple BCCs at an early age but it is not associated with development of large numbers of BCCs. The amount of UV light person receives appears to be more important.