Diamond–Blackfan anemia syndrome (DBAS) and other inherited ribosomal disorders represent a unique subgroup of ribosomopathies with hematological manifestations, particularly erythroblastopenia and transfusion-dependent anemia. DBAS encompasses classic ribosomal protein gene mutations and a subset termed “DBA-other,” associated with mutations in genes such as GATA1 and TP53. Diagnosis hinges on genotype–phenotype correlation, with increasing detection in nonclassical presentations. Management includes corticosteroids, red blood cell (RBC) transfusions with iron chelation, and hematopoietic stem cell transplantation (HSCT) for steroid-refractory cases. DBAS carries a significant risk of clonal evolution and solid tumors, such as osteosarcoma and colorectal cancer, necessitating long-term surveillance. The chapter also addresses cartilage-hair hypoplasia (CHH), another ribosomopathy with overlapping anemia and immunodeficiency phenotypes, emphasizing supportive care and HSCT in severe cases. As treatment strategies evolve, including gene therapy and targeted agents, optimal management requires expert multidisciplinary care and adherence to updated international guidelines.