Typical signs and symptoms in the early phase of the classic phenotype are acroparesthesia, episodes of diarrhea and abdominal pain, cornea verticillata, dyshidrosis, tinnitus, angiokeratomas, and microalbuminuria [1]. Severe organ manifestations become evident during the third decade of life in males and approximately the fourth decade of life in females [2,3]. Cardiac hypertrophy, renal dysfunction, and stroke are the most severe complications [4].