Historically, the first recorded case of severe hypoplasminogenemia with xylem conjunctivitis and obstructive hydrocephalus was in 1994 and involved an 18-month-old girl from Turkey, while since then the total number of reported cases worldwide does not exceed 20 [10, 12]. CNS involvement in plasminogen deficiency is unusual because the blood-brain barrier restricts plasminogen entry, the CNS has alternative fibrin-clearance pathways, and typical fibrin-rich pseudomembranes form on mucosal, not neural, surfaces. Here, PLG is linked to Hydrocephalus.