Paroxysmal Cold Haemoglobinuria (PCH) is considered the rarest form of autoimmune haemolytic anaemia (AIHA), although it is likely underdiagnosed. It is caused by biphasic haemolysins, known as Donath-Landsteiner antibodies, which bind to erythrocytes at 4°C and trigger complement-mediated lysis at 37°C. In the paediatric population, PCH is typically preceded by an upper respiratory tract infection caused by common pathogens. The resulting intravascular haemolysis often leads to acute and severe anaemia, which is usually self-limiting. Diagnosis requires performing the Donath-Landsteiner test in cases of AIHA with a direct antiglobulin test (DAT) positive for C3, in the presence of a clinical history suggestive of PCH. Treatment is mainly supportive and transfusions are generally well tolerated. Most of the available data on PCH derive from case reports and case series, with a lack of large-scale studies. The paper presents two cases diagnosed over the past three years and compares their diagnoses and managements with the existing literature.