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Lipoblastoma usually happens in youth and is related to rearrangements associated with the PLAG1 gene. We present an individual with an isolated mass considered a lipoblastoma clinically, radiographically, and histologically. The lesion was diagnosed as a PIK3CA-adipose lesion following the structure had been unfavorable for PLAG1 rearrangement and included a somatic PIK3CA mutation (H1047R). Although PIK3CA alternatives are associated with POSITIVES (PIK3CA-related overgrowth range)