Study of consanguineous marriages, and their clinical consequences have an important place in genetics. Consanguineous marriage is a major feature of the family system in Iran as well as most eastern societies. Regarding the high prevalence of consanguineous unions in most of the Iranian population and inbreeding as an important risk factor for genetic abnormalities, it is necessary to pay attention to some genetic conditions, which are more frequent in progenies of related parents. Statistical analysis of frequencies of various defects among progenies of consanguineous and unconsanguineous marriages is the main purpose of this study. Twenty-five hundred files of consultants from the genetic counseling center in Shiraz from 2000 to 2003 have been included in this study. According to the diagnosis of genetic counselors and drawn pedigrees, different abnormalities and degrees of consanguinity were specified. Chi-square statistical test was used to detect significant differences of frequencies. The average of consanguineous marriages was 86 and 76.5 percent of the marriages were among first cousins. The most frequent registered genetic conditions, out of 6900 detected conditions, were mental retardation (842 cases), deafness (440 cases), eye abnormalities (215 cases), paralysis (196 cases), frequent abortions (190 cases), beta thalassemia (157 cases), mental retardation together with congenital abnormalities (155 cases), hearing impairment (148 cases), Down syndrome (145 cases), epilepsy (144 cases), and congenital heart defects (140 cases). By analysis of frequencies, mental retardation, deafness, eye abnormalities, frequent abortions, paralysis, physical and mental retardation, congenital malformations and abnormalities, infantile and neonatal deaths, low IQ, developmental delay, mental retardation with paralysis, skeletal defects, microcephaly and mental retardation together with microcephaly were more frequent among progenies of consanguineous parents than unconsanguineous parents (p<0.05). These findings imply the potential risk of consanguineous marriage in the occurrence and recurrence of 14 types of abnormalities. Regarding the high prevalence of consanguineous marriages, not only in the consultants but also in the general population, abnormalities with low frequencies are nonrandom and take place in special subpopulations. In contrast, abnormalities with high frequencies such as mental retardation and hearing loss are genetically more heterogeneous in the population.