Extrarenal involvements were uncommon and only seen in nine reported cases, and symptoms included seizure (n = 2), brain development retardation (n = 1), arrhythmia (n = 1), vesicoureteral reflux (n = 1), ovarian cyst (n = 1), macula retinitis (n = 1), low serum C3 level (n = 1), and cataract (n = 1). Here, C3 is linked to Ovarian cyst.