Aim: To create awareness about rare clotting disorders in children and to highlight the different dental treatment approaches that can be used while planning the management in such cases. Background: A prerequisite for successful wound healing is achieving good hemostasis by effective vascular spasm, platelet plug formation, and finally blood coagulation. In the general population, postoperative bleeding after dental treatment is self-limiting. However, a certain segment of the population does suffer from inherited bleeding and clotting disorders, wherein standard invasive dental procedures can trigger bleeding episodes, which could be life-threatening in absence of the requisite precautionary measures being followed. Case description: One such condition is congenital factor XIII deficiency, a rare autosomal recessive disease usually associated with early onset of serious or life-threatening bleeding episodes, such as, intracranial hemorrhage or umbilical cord bleeding. This case series details the complete dental management of three children suffering from factor XIII deficiency. Conclusion: Factor XIII is a rare coagulation cascade, and by this case series, complete dental treatment varying from noninvasive to be kept under observation to invasive dental extraction and fracture reduction was carried out with the hematologists consultations. Clinical significance: This case series creates awareness about this rare condition and the need for a multidisciplinary approach involving dentists and hematologists in the effective management of such patients.