Background <p>Podocyte infolding glomerulopathy (PIG) is a rare kidney disorder characterized by the infolding of podocyte processes into the glomerular basement membrane. The condition presents with nephrotic syndrome, yet its pathogenesis and clinical implications remain poorly understood due to limited case reports. Most documented cases have originated from Japan, highlighting a significant gap in the literature regarding its prevalence and characteristics in other regions, including the Indian subcontinent.</p> Case <p>A 5-year-old boy presented with hypertension, edema, nephrotic range proteinuria, and 3 weeks later developed macroscopic hematuria. Kidney biopsy revealed characteristic microtubule formation within the glomerular basement membrane, confirming the diagnosis of PIG. Initial treatment with prednisolone was unsuccessful, leading to the addition of tacrolimus which resulted in significant improvements in the child’s proteinuria and hematuria.</p> Conclusion <p>This case contributes to the limited literature on PIG and highlights the need for more research to establish standardized treatment protocols.</p>