Constitutional bone marrow failure (BMF) syndromes caused by immune dysregulation represent a growing subset of hematologic disorders, characterized by complex interactions between hematopoiesis and immune dysfunction. Unlike classical BMF syndromes, these disorders often present with overlapping features of cytopenias, autoimmunity, and immunodeficiency, frequently linked to underlying genetic mutations affecting immune regulatory pathways. Conditions such as GATA2 deficiency, adenosine deaminase 2 (ADA2) deficiency (deficiency of adenosine deaminase 2 (DADA2)), and DNA double-strand break (DSB) repair disorders exemplify this spectrum, with marrow failure arising from both intrinsic stem cell defects and extrinsic immune-mediated destruction. Diagnostic evaluation requires a comprehensive immunologic and genetic workup, including next-generation sequencing and biomarker profiling. Early identification is essential for therapeutic decision-making, particularly in guiding hematopoietic stem cell transplantation (HSCT), the only curative option for many of these disorders. This chapter provides an in-depth overview of the pathophysiology, diagnostic approach, and evolving management strategies for BMF syndromes associated with immune dysregulation.