The synchronous presentation of venolymphatic anomalies of the orbit and noncontiguous intracranial cavernous malformations is uncommon. Herein, we present a case of an 11-month-old female patient diagnosed with orbital venolymphatic anomaly associated with a large cavernous malformation in the posterior fossa, who underwent complete surgical resection of the latter. The immunohistochemical analysis was positive for podoplanin, a marker expressed by lymphatic endothelial cells, but not vascular endothelium. This exceptional finding suggests lymphatic involvement in the etiology of the lesion. In our review of the literature, we did not find similar cases in patients under 1 year of age.