Autoimmune haemolytic anaemia (AIHA) is a well-recognised complication of lymphoproliferative disorders, and has been reported in association with all B and T cell non-Hodgkin lymphoma subtypes with the exception of mantle cell lymphoma (MCL). We describe herein a case of MCL diagnosed in an initially asymptomatic 66-year-old woman who developed transfusion-dependent AIHA 6 months later coincident with lymphoma progression. The AIHA failed to respond satisfactorily to conventional treatment (high-dose oral prednisolone) but rapidly resolved following commencement of non-rituximab-containing combination chemotherapy in parallel with complete remission of the lymphoma. This is the first of such cases to be described in the literature and confirms that the immune environment of MCL can predispose to AIHA in the same way as in other lymphoma subtypes. Despite this being an infrequent occurrence, clinicians should be aware that AIHA is a potential complication of MCL and may be more successfully controlled by treating the underlying lymphoma rather than relying on conventional anti-haemolytic strategies such as steroids.