Context: Inherited errors of immunity (IEI) or primary immunodeficiencies are a group of genetic rare diseases predisposing to severe infections, autoimmunity, and malignancies. The overall risk for malignancies in pediatric IEI-patients is estimated to range from 4–25%, of which 60% of cases are of hematological origin. Objective: Evaluation of hematological malignancies in pediatrics IEI. Methods: Were included 13 patients 3 of which developed hematological malignancies and were evaluated for demographics, clinical features, and prognosis. Results: The prevalence of hematological malignancies among our patients was 23% (3/13). The male-to-female ratio was 2:1. The mean age at diagnosis of malignancy was 8.6 years (range 5-14). Non-Hodgkin lymphoma was the malignancy type diagnosed in all the pediatric patients (n=3, of which Wiskott-Aldrich-1, ataxia-telangiectasia-1, common variable immunodeficiency (CVID)-1. However, 2 patients (52.5%) died (1-during first line-chemotherapy, 1-NHL was established postmortem). 1 patient underwent bone marrow transplantation and is currently under supervision. Conclusions: In this study, we related an association between hematological malignancies and IEI. Lymphoid malignancies were the most common malignancy, this warns of the need for multidisciplinary monitoring of these patients.