Desmoplastic infantile astrocytomas (DIAs), are rare supratentorial tumors, usually observed in the first 24 months of life. Despite their aggressive appearance, they tend to follow a favorable clinical course. Total or near total resection of tumor is usually the treatment option. Desmoplastic Infantile Ganglioglioma (DIG) and DIA are WHO grade I tumors that have similar clinical and morphological findings. The only criterion in differential diagnosis is the neural component of DIG. These tumors both have dense fibroblastic stroma and positive staining with glial fibrillar acidic protein (GFAP) and CD34. A rare case of desmoplastic infantile astrocytoma presenting with right side partial seizures presented in a 1-year-old child. A rare case of desmoplastic infantile astrocytoma presenting with focal onset generalized seizures presented in a 1-year-old child. Despite their radiological and histological properties, these tumors have a benign course. After 3-year follow-up for the first case and 1-year follow-up for the second case, there was no recurrence.