C1q nephropathy is a rare cause of idiopathic nephrotic syndrome seen in children and young adults. Mesangial proliferation with deposition of C1q in the mesangium on immunofluorescence examination is the main pathological feature of the disease. Diagnosis is made by ruling out systemic lupus erythematosus with clinical and laboratory investigation. In this report, we discussed a patient presenting with nephrotic syndrome and C1q nephropathy with a review of the relevant literature.