We report the association of partial lipodystrophy (PL) and nephritis in an 11-year-old boy. He had symmetric absence of facial fat with retention of adipose tissue in the arms, chest, abdomen and hips associated with macroscopic hematuria, proteinuria combined with depression of the complement C3 level. The patient had rapidly progressive glomerulonephritis (RPGN) and the histopathological study showed findings of mesangio-capillary glomerulonephritis (MCGN) and crescents. We managed and followed up this patient for three years. At the last follow-up visit, he still has mild proteinuria and microscopic hematuria and stable renal function.