Introduction: Progressive Familial Intrahepatic Cholestasis (PFIC) comprises a rare group of autosomal recessive disorders characterized by defective bile secretion, leading to progressive cholestasis, liver injury , and eventually cirrhosis. PFIC Type 10 is a recently identified subtype caused by pathogenic variants in the MYO5B gene, which disrupts the integrity of tight junctions in the biliary canalicular membrane. Due to its rarity , clinical recognition remains a significant challenge, particularly in South Asia. Case presentation: We report a case of an 11-month-old female infant, born to nonconsanguineousparents, presenting with a three-month history of progressive icterus, acholic stools, and intractable pruritus. Physical examination revealed significant growth failure and isolated hepatomegaly . Laboratory investigations demonstrated conjugatedhyperbilirubinemia, elevated transaminases, and prolonged PT/INR, but characteristically normal gamma-glutamyl transferase (GGT) levels. Ultrasound and CECT imaging showed hepatomegaly and a gallbladder neck calculus without ductal dilatation. Liver biopsy revealed features of evolving cirrhosis. Given the ""normal-GGT cholestasis"" phenotype, whole-exomesequencing was performed, identifying a homozygous pathogenic variant in the MYO5B gene (c.1532A>T), confirming PFIC10. Management included ursodeoxycholic acid, cholestyramine, antihistamines, and fat-soluble vitamin supplementation alongside specialized nutritionalsupport. Due to the progressive nature of the disease the patient remains undermultidisci plinary surveillance for potential biliary diversion surgery or liver transplantation. Conclusion: PFIC10 is a rare but critical diagnosis to consider in pediatric patients with normalGGT cholestasis. It highlights the important role of genetic testing in achieving an accurate diagnosis as early identification is vital for managing complications, providing cascade screening, counseling, and planning long-term surgical interventions.