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Inborn errors of immunity (IEIs) comprise a broader spectrum of manifestations than primary immunodeficiencies (PID). They often present with immune dysregulation manifestations such as lymphoproliferation (LP), a common yet underrecognized hallmark of IEIs that encompasses disorders ranging from chronic benign lymphadenopathy to malignant lymphoid neoplasms. Given the difficulties in diagnosing these conditions, including their heterogeneous presentation, this narrative review aims to increase awareness of IEIs by highlighting LP as a key clinical indicator, particularly in hematology-oncology settings. We present clinical, histopathological, and radiological features-especially those related to LP-that should prompt suspicion of IEIs. Additionally, we advocate for a multidisciplinary approach, fostering effective communication among all potentially involved specialists (clinical immunologists, hematologists, oncologists, radiologists, geneticists, and pathologists) to facilitate accurate, timely disease recognition. Early genetic diagnosis in IEIs is crucial for implementing genetic counseling, targeted therapies, and follow-up protocols, ultimately affecting patient outcomes and quality of life.