Introduction: Griscelli syndrome is a rare autosomal recessive disorder characterized by partial albinism, immunodeficiency , cytopenias, and an increased risk of hemophagocytic lymphohistiocytosis (HLH). Three subtypes have been identified, of which Griscelli syndrome type 2 is the most severe, associated with recurrent infections and life-threatening immune dysregulation. Here reported a child with Griscelli syndrome type 2 presenting with recurrent infections and neutropenic sepsis. Case Report: A 3-year-old girl, the first child of healthy consanguineous parents, presented with recurrent upper respiratory tract infections, abdominal distension, and features of partial albinism. There was no significant past history of allergies, blood transfusions, or immunizationrelated issues. On examination, she had severe pallor, ecchymotic patches, generalized lymphadenopathy , and marked hepatosplenomegaly . Full blood count revealed leukopenia with significant neutropenia and the blood picture showed pancytopenia with predominant neutropenia. Inflammatory markers and liver function tests were elevated. Hair shaft microscopic examination confirmed the diagnosis of Griscelli syndrome while further evaluation with bone marrow biopsy revealed acute lymphoblastic leukemia. The child was subsequently transferred to the Apeksha Hospital, Maharagama, for specialized management. Discussion: Griscelli syndrome type 2 is a life-threatening condition that may remain undiagnosed due to limited access to genetic testing. Early clinical recognition is crucial, as hematopoietic stem cell transplantation remains the only curative treatment and is most effective when performed before disease progression and severe complications occur.