Sphingosine-1-phosphate lyase insufficiency syndrome (also known as SPLIS, renal, endocrine, neurological and immune (RENI) syndrome, SGPL1 deficiency, nephrotic syndrome type 14, NPHS14, steroid-resistant nephrotic syndrome type 14, and familial steroid-resistant nephrotic syndrome with adrenal insufficiency) is an inborn error of metabolism (OMIM # 617575) associated with kidney, endocrine, immunological, neurological, and skin manifestations [1]. The gene discussed is SGPL1; the disease is Adrenal insufficiency.