Lipoblastoma is an uncommon benign soft tissue tumor arising from embryonal mesenchymal cells. It occurs mostly during early childhood with a predilection for the extremities, trunk, head and neck. This tumor tends to spread locally and no metastatic potential has been reported. Complete surgical resection is mandatory to prevent local recurrence. MRI provides excellent presurgical delineation of the tumor and confirms its fatty nature. Histology demonstrates the presence of lipoblasts in different stages of maturation; cytogenetic evaluation often discloses chromosomal anomalies of tumoral cells. A case of lipoblastoma of the buttock in a 10 month infant associated with anomalies of chromosome 8 is reported.