AbstractThe rare inherited coagulation disorders (RICD) are uncommon and thus not well‐defined in terms of severity or management. Inheritance is autosomal; in some of these disorders in the heterozygote state affected individuals may be mildly symptomatic. Severe deficiencies are more common in association with consanguinity. Factor X and factor XIII deficiency have the most severe manifestations, while factor XI deficiency is the least severe. Factor VII and factor XI deficiencies show a poor relationship between the factor level and bleeding risk. Unlike hemophilia, women are equally affected by these RICD and can have problems related to menstruation and childbirth. Pediatr Blood Cancer 2012; 60: S37–S40. © 2012 Wiley Periodicals, Inc.