Vascular anomalies of the inner ear have been documented in only a few isolated case reports. The goal of our study was to describe, qualify and quantify vascular variations of the inner ear in 122 temporal bones from 64 pediatric subjects aged between 0 and 10 years. The average age was 11.6 months. Horizontal sections of the temporal bone, examined by light microscopy, revealed vessels coursing freely through the perilymphatic space of the cochlea, especially in the apical turn. Other findings included abnormally wide vessels in the stria vascularis as well as a vascular malformation of the internal auditory canal. Our study demonstrated more atypical vessels in the cochlea than in the vestibular labyrinth. We found a statistically significant positive correlation between vascular variations of the inner ear and concomitant cardiac anomalies or endolymphatic hydrops. We also discuss the possible etiology and potential significance of these findings in terms of disturbances of the function of the inner ear.