Congenital plasminogen deficiency is an infrequent disorder, which usually becomes symptomatic as ligneous conjunctivitis. However, pseudomembranous lesions in the mucosa of the pharynx, tracheobronchial tree, and the peritoneum may likewise occur. An accompanying hydrocephalus is extremely rare; only 16 cases have been reported to date. The reports indicate that hydrocephalus, even if treated by ventriculoperitoneal (VP) cerebrospinal fluid (CSF) shunting, worsens the prognosis substantially. Thus, VP CSF shunting does not seem to be the optimal therapy for hydrocephalic children with plasminogen deficiency. We add two cases to the literature, and, on the base of our experience, we propose a management strategy for the hydrocephalus. We report the case history of two children with plasminogen deficiency and associated hydrocephalus. Both children initially were treated with VP shunts and had a very similar clinical course with multiple shunt malfunctions due to nonabsorption by the peritoneum. In the first child, the attempt to treat the hydrocephalus with a ventriculoatrial (VA) shunt failed due to catheter thrombosis. Finally, a ventriculocholecystic shunt was placed in both children, which worked well. In patients with plasminogem deficiency and associated hydrocephalus, special care must be taken in the management of hydrocephalus. The absorptive capacity of the peritoneum is reduced by pseudomembrane formation, which results in VP shunt malfunction. The plasminogen deficiency results in early thrombus formation if atrial catheters are used. Therefore, the authors believe that ventriculocholecystic shunting should be considered early on in the course of the disease.