Desmoplastic infantile gangliogliomas (DIGs) are rare intracranial, cystic tumors, usually detected within the first 2 years of life. Histologically, these tumors are characterized by intense desmoplasia and a divergent astrocytic and neuronal differentiation. Less than 60 well-documented cases of this extremely rare tumor are reported in the literature. We present the case of a 10-month-old male child presenting with a large, cystic, intracranial lesion and having the characteristic histological features of a DIG. In addition, the lesion also showed focal areas of calcification within the tumor, not usually considered a feature of this entity. The present case extends the reported spectrum of this rare entity and describes the unusual occurrence of calcification within the lesion.