Introduction: Wolman disease is a rare, severe autosomal recessive lysosomal storage disorder caused by deficiency of lysosomal acid lipase due to pathogenic variants in the LIPA gene. The estimated global incidence is less than 1 in 100,000 live births. It typically presents in early infancy with progressive hepatosplenomegaly , adrenal calcifications, cholestatic jaundice, dyslipidemia, and rapid clinical deterioration. Without treatment, it is usually fatal within the first year of life. Case presentation: A two-month-old infant born to second-degree consanguineous parents presented with prolonged jaundice, pallor, and progressive abdominal distension. There were no significant gastrointestinal or metabolic symptoms at onset. Examination revealed deep icterus and massive, firm hepatosplenomegaly . Laboratory evaluation showed pancytopenia,deranged liver function tests, and an atherogenic lipid profile. Abdominal imaging demonstrated bilateral adrenal calcifications. Bone marrow examination revealed foamy histiocytes, while liver biopsy showed micronodular cirrhosis. Based on clinical, biochemical, and radiological findings, a presumptive diagnosis of Wolman disease was made. However, confirmatory enzyme assay and molecular testing were not performed due to financial constraints. Despite supportive management, the baby succumbed within one week of hospitalization. Subsequently , a sibling presented with a similar phenotype, and genetic testing confirmed a homozygous 63-bp deletion in exon 6 of the LIPA gene (Chr10(GRCh37): g.90984849_90984911del). Discussion: This represents the first reported novel LIPA variant causing Wolman disease from Sri Lanka. The case highlights the importance of early clinical suspicion, access to genetic testing, and appropriate genetic counseling, particularly in consanguineous families.