Background: Factor XIII deficiency is a rare bleeding disorder that presents various life threatening clinical manifestations such as central nervous system (CNS) and umbilical cord bleeding. Thisdisorder occurs due to different mutations and polymorphisms among which Val34Leu has associated with the majority of clinical features.

Objectives: The current study aimed to evaluate the relationship between polymorphism and umbilical cord bleeding as the most common clinical feature in affected patients with FXIII deficiency in Sistan and Baluchistan province, Iran.

Patients and Methods: This case control study was conducted on 12 FXIII deficient patients with umbilical cord bleeding as the case group and 15 patients with FXIII deficiency without umbilical cord bleeding as the control group. All the patients and controls were evaluated for Val34Leu polymorphism. Eventually, the obtained data were analyzed by SPSS software version 19.

Results: Results of the current study showed no statistically significant association between FXIII Val34Leu and umbilical cord bleeding. In addition no statistically significant difference was found between the cases and the controls.

Conclusions: FXIII Val 34Leu polymorphism is not associated with umbilical cord bleeding.