Background: Epidermoid cysts may remain asymptomatic for a long period of time due to their slowly growing pattern corresponding with the normal human skin turnover time and due to soft and light cyst content. They do not cause compression of neural and vascular structures initially that is why almost all of the cases in the literature are diagnosed during adulthood. Methods: We report here an epidermoid cyst in childhood, which was located in the medulla oblongata, unusually and atypically with liquefied cyst content. The liquefaction may occur due to an intrauterine or early childhood infection. The reported case also suffered previously a severe respiratory infection. Although the cyst is located in and around a highly eloquent neural area, plasticity of the brain stem prevented neurological deterioration due to this very slow growing extraaxial mass lesion. The ordinary cyst content found in the center of the cyst cavity during the operation suggested that the same ordinary material, which was previously at the periphery, ran to get liquefied in time. Conclusion: We suggest that the symptoms of this present case appeared very early due to liquefaction of the cyst content with compression and displacement of the brain stem caudally. The recent infection process may predispose the pathological condition.