Nephrotic syndrome in children with visceral leishmaniasis (VL) is an uncommon presentation, often accompanied by other clinical signs such as febrile splenomegaly. This report highlights two cases of VL in immunocompetent children admitted to Mohamed VI University Hospital in Marrakech, who developed nephrotic syndrome as a complication. One child unfortunately passed away, while the other responded well to treatment with corticosteroids and pentavalent antimonial drugs, showing favorable clinical progress. This rare condition underscores the need to raise awareness among clinicians to facilitate early diagnosis and prompt treatment.