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This case report presents a novel finding of Rectosigmoid Hirschsprung disease in association with Factor VII deficiency in 2 years female presented to King Abdulaziz University Hospital (KAUH). She was admitted in pediatric surgery unit. On examination; she looked ill and there were signs of infection at the colostomy site. The infection was treated and her overall health improved in 2 months. Mixing study and Factor VII level confirmed the presence of Factor VII deficiency. Congenital Factor VII deficiency is a rare autosomal recessive disease occurring at a rate of 1:500,000 individuals while Hirschsprung disease (HD) is found in 1:5000 individuals. The combination of these two disorders is extremely rare. The patient was successfully managed for both disorders and discharged in good health in stable condition with no hemorrhagic complication. This case report describes an exceptionally unusual combination of congenital factor VII deficiency and Hirschsprung Disease in same patient. [ABSTRACT FROM AUTHOR]