Renal agenesis is considered as a signature phenotype of KAL1 mutations and can be used as an early marker for genetic screening.[3] In this article, we for the first time reported a diagnosis of KS in a 19-year-old young man with 8 rare urinary disorders demonstrating a conservative change in the KAL1 gene (c.1600G > A, p. Val534Ile) and the PROKR2 gene (c.533G > A, p. Trp178Ser) in this article. The gene discussed is ANOS1; the disease is renal agenesis.