Introduction Recent advances in immunologic techniques have lead to increase recognition of primary immunodeficiency diseases (PID). Objective We sought to analyze and describe the spectrum of PID at a tertiary care center in Morocco. Methods A retrospective study from 2011 to 2017 was conducted on 27 children diagnosed with PID. Result Over the study period, combined immunodeficiencies with associated or syndromic features were the most common category (48.15%) followed by immunodeficiencies affecting cellular and humoral immunity (25.9%), congenital defects of phagocyte number or function (14.81%), predominantly anti-body deficiencies (7.4%), and diseases of immune dysregulation (3.7%). The most frequent disorder was Ataxia-telangiectasia (44.4%). The mean age at diagnosis was 4.62 years. The consanguinity rate was 74.1%. The principal clinical signs were lower respiratory tract infections (59.2%), neurological manifes-tations (44.4%), failure to thrive (51.8%) and skin infections (33.3%). Two patients who have immunodeficiencies affecting cellular and humoral immunity died. Conclusion This study indicates that PIDs are not rare in Morocco and that combined immunodeficiencies with associated or syndromic features are the most common category. Future research should focus on identifying gene defect for PID patients.