Mohammad Firoz Anjum,1 Suraj Sah,2Inesh Khanal,2 Sharmila Gainju,3Sriram KC41Pediatric Nephrologist, Patan Academy of Health Sciences, Lagankhel, Lalitpur, Nepal, 2Intern Doctor, Patan Academy of Health Sciences, Lagankhel, Lalitpur, Nepal,3Resident, Department of Pediatrics, Patan Academy of Health Sciences, Lagankhel, Lalitpur, Nepal,4Pediatrician, Armed Police Force Hospital, Balambu, Chandragiri, Kathmandu, NepalCorrespondence: Dr Inesh Khanal, Intern Doctor, Patan Academy of Health Sciences, Lagankhel, Lalitpur, Nepal, Email: std.ineshkhanal@pahs.edu.np , Phone: +977-9813865449.Author contribution:Dr. Firoz Anjum: Conceptualization; Investigation; Methodology; Project administration; Software; Supervision; Validation; Writing - original draft; Writing - review & editing.Dr. Suraj Sah: Conceptualization; Methodology; Writing - original draft; Writing - review & editingDr. Inesh Khanal: Conceptualization; Formal analysis; Software; Supervision; Writing - original draft; Writing - review & editingDr. Sharmila Gainju: Conceptualization; Methodology; Project administration Supervision; Writing - review & editingDr. Sri Ram KC: Conceptualization; Investigation; Supervision; Writing - original draft; Writing - review & editingAbstractLupus nephritis is a severe manifestation of systemic lupus erythematosus (SLE) typically characterized by glomerular “full-house” immunofluorescence. However, non-lupus nephropathies may occasionally exhibit similar patterns, creating diagnostic uncertainty. Non-lupus full-house nephropathy (FHN) is a recently recognized entity in which a full-house immunofluorescence pattern occurs in the absence of serological or clinical evidence of SLE. We report a 4-year-old girl who presented with generalized edema, hematuria, hypertension, and nephrotic-range proteinuria. Laboratory evaluation revealed anemia, elevated serum creatinine, and low complement C3 levels but negative antinuclear antibody (ANA) and anti-double-stranded DNA (anti-dsDNA) tests. Renal biopsy demonstrated diffuse proliferative glomerulonephritis consistent with lupus nephritis Class IV, with full-house immunofluorescence positivity for IgG, IgA, IgM, C3, and C1q. In the absence of systemic or serologic lupus features, a diagnosis of non-lupus full-house nephropathy was made. The patient was treated with pulse methylprednisolone followed by oral prednisolone, monthly cyclophosphamide for six months, and maintenance therapy with azathioprine and low-dose steroids. Hydroxychloroquine and antihypertensive agents were added. She achieved clinical and biochemical remission on follow-up.Keywords: Non-lupus full-house nephropathy, Lupus nephritis, Seronegative lupus, Pediatric nephrology, Diagnostic dilemma