Background: Griscelli Syndrome (GS) type 2 is a rare autosomal recessive disease caused by mutations in the RAB27A gene. Griscelli syndrome is of three types with GS type 2 being the most common variety. It is primarily characterized by a combination of partial albinism, Hemophagocytic Lymphohistiocytosis (HLH), and neurological impairment. GS 2 is mostly lethal unless an allogeneic Peripheral Blood Stem Cell (PBSC) transplantation is performed in time. Case Presentation: We hereby describe a case of GS type 2 in a 3-year-old boy who presented with central nervous involvement and primary HLH. He was also a post-COVID-19 recovered case and had been treated for Cytomegalovirus (CMV) reactivation. The patient was first managed for primary HLH following HLH-2004 protocol using a combination of dexamethasone and etoposide followed by a haploidentical peripheral blood stem cell transplantation and has shown a good response with complete donor chimerism. Conclusion: PBSC transplantation in GS when performed promptly with proper care and support can be successful in providing the patient with a new lease of life.
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