A maternally-inherited frameshift variant in SALL1 was identified in an ARM-PLUS patient with a perineal fistula, atrial septal defect, agenesis of the septum pellucidum, right single transverse palmar crease, flat feet, and postnatal cerebral hemorrhage leading to hydrocephalus (ID = 2). The gene discussed is SALL1; the disease is Hydrocephalus.