Desmoplastic infantile ganglioglioma (DIG) is rare uncommon intracranial brain tumor of infancy comprises of 0.5-1.0% of all the intracranial tumors. DIGs are commonly located in supratentorial region superficially with more affection to the frontal and parietal lobes and have voluminous size with both cystic and solid components. Histologically it is mixed glial and neuronal brain tumor and showed intense desmoplastic reaction at the periphery of the tumor and dural attachment. DIGs are most benign intracranial tumors and labeled as World Health Organization (WHO) grade-I. Data available from the literature suggest that surgical resection is the mainstay of treatment and no chemotherapy or radiotherapy is indicated if complete resection of the tumor has been achieved. In general, DIG has good prognosis and recurrence free intervals of up to 14 years have been reported. In our case report, a-12-month old girl presented to emergency department with history of first seizure of her life. Seizure duration was less than a minute, focal type and confined to mouth and left upper limb. Magnetic resonance imaging (MRI) brain with gadolinium showed diffuse right frontoparietal localized mass with well-defined right temporal cyst measuring 4×3×2.7 cm without mural nodule and gadolinium enhancement. There was extensive dural thickening and enhancement seen in the anterior temporal region on post contrast. Electroencephalogram was normal. Patient underwent right craniotomy and gross total excision of tumor done. Frozen section and final histology reported DIG (WHO grade-I).