LRBA (LPS responsive beige-like anchor protein) is a critical regulator of immune homeostasis and vesicle trafficking. The protein functions in coupling signal transduction and vesicle trafficking to enable polarized secretion of immune effector molecules, and plays a role in mitophagy by regulating ATG9A trafficking to mitochondria 1. LRBA deficiency causes a primary immunodeficiency characterized by recurrent infections, autoimmunity, and lymphoproliferation 2. Patients with LRBA deficiency present with life-threatening infections, pneumonia, organomegaly, autoimmune enteropathy, and growth failure more frequently than those with similar conditions like CTLA-4 haploinsufficiency 2. The condition is associated with monogenic inflammatory bowel disease, often presenting before age 6, with substantial extraintestinal complications including atypical infections, dermatologic abnormalities, and autoimmunity 3. Flow cytometry can aid in diagnosis by detecting reduced LRBA protein expression 4. Treatment options include abatacept, which shows superior long-term disease control compared to conventional immunosuppressants, with 79.3% of patients achieving sustained complete control 5. Hematopoietic stem cell transplantation remains a curative option for severe cases.