This document presents the case of a 5-year-old male child diagnosed with Hyper IgE overlap syndrome associated with Prolidase enzyme deficiency. The child has a history of recurrent ear discharge and skin lesions since infancy. Examination found multiple healed skin lesions, enlarged lymph nodes, and an enlarged spleen. Testing found very high IgE levels and genetic testing confirmed Prolidase deficiency. This rare condition combines features of both Hyper IgE syndrome and Prolidase deficiency, caused by mutations in the PEPD gene. Prolidase deficiency is typically associated with skin fragility, infections, and developmental delays.