We report two cases of lipoblastoma of the buttock in a 10-month-old boy and a 20-month-old girl, the first with rearrangement of chromosome 8 and the second without cytogenetic abnormality, and one case of lipoblastomatosis of a leg in a 6-month-old boy with a normal karyotype but with a rearrangement of the PLAG1 gene. Lipoblastoma and lipoblastomatosis are two different presentation of the same rare benign soft tissue mesenchymal tumour arising from fetal white fat and occurring almost exclusively in young children under 3 years. These neoplasms have no malignant potential but may recur in cases of incomplete resection. Histological diagnosis sometimes used to be difficult because of the close resemblance of the lesion with myxoïd liposarcoma. Nowadays, cytogenetic analysis may contribute to the diagnosis by showing abnormalities of the long arm of chromosome 8, leading to rearrangement of the PLAG1 gene. Actual advances in cytogenetic molecular analysis may aid in accurate diagnosis.