Background: Microcephaly is a congenital disorder characterized by a head circumference falling more than two standard deviations below the mean with respect to age- and gender-matched population sample, which is divided into isolated and syndromic types. This study aimed to show the inheritance pattern of microcephaly and role of the consanguineous marriage in Southwestern Iran.

Patients and Methods: In this study, medical records of 1202 people (out of 3951 families, 30.4%) with autosomal recessive pattern inherited disorder who attended the centers of Khuzestan state welfare organization, southwestern Iran during 2011 to 2016 for genetic counseling, were reviewed.

Results: Overall, out of 1202 cases of microcephaly, 114 (9.48%, 2.3/100,000 live births) people were detected. The results showed that most patients were female (66.7%), from Arab ethnicity (63%). Most of the patient‘s fathers were at the level of primary school education (63%) and self-employed (63%), while patient‘s mothers completed primary school education (37%) or illiterate (37%) and housewife (100%). Income distribution showed that the majority of families were below the poverty line (63%), and lived in the urban area (37%). The study showed that the frequency of consanguineous marriage was much higher (81.5% vs. 18.5%, P= 0.0013).

Conclusion: In as much as lack of access to early medical attention, poor socio-economic state and harsh environmental factors affect mental health response, these factors are believed play crucial role in reducing the incidence of mental health and associated abnormalities. Considering the relatively high prevalence of inherited microcephaly, it is important to set up clinical guidelines for early detection and management of these conditions to decrease the associated morbidity and mortality. [ Ethiop. J. Health Dev. 2017;31(2):119-123]