Introduction: Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by impaired B-cell differentiation. Type 8, caused by mutations in theLRBA gene, is associated with significant immune dysregulation and autoimmunity . Seronegative post-infantile giant cell hepatitis is a rare hepatic manifestation in this context. Case presentation: A previously healthy 7-year-old boy presented with a history of jaundice,vomiting, and epistaxis. He was the T1 of twins born to consanguineous parents. On examination, he had pallor, icterus, and hepatosplenomegaly . investigations revealed pancytopenia with reticulocytosis and direct hyperbilirubinemia. The direct antiglobulin test was initially positive . Viral screening was negative. Thalassaemia and Wilson disease screening were normal. CT abdomen demonstrated hepatosplenomegaly without evidence of chronic liver disease. no portal hypertension either.Liver histology is consistent with post-infantile giant cell hepatitis. Autoimmune hepatitis antibody panel was negative. Serum immunoglobulin levels were within normal limits. Bone marrow biopsy excluded malignancy .Despite blood , platelets transfusions and intravenous im munoglobulin therapy , the patient deteriorated. Administration of intravenous methylprednisolone followed by oral prednisoloneled to marked improvement in cytopenias and liver function tests. A relapse occurred during steroid tapering in association with a viral infection but resolved along with recovery from the infection.Whole exome sequencing identified an autosomal recessive pathogenic LRBA gene variant, confirming CVID type 8. Conclusion: This case highlights Early recognition of immune dysregulation and timely initiation of immunosuppressive therapy were crucial in achieving remission.