A pseudo-tail is defined as a tail-like lesion in the lumbosacrococcygeal region that is not a true tail but one caused by disease. Perineal lipoma is one of the conditions that may present as a pseudo-tail. Congenital perineal lipoma is a rare disease and in particular, isolated congenital perineal lipoma without other anomalies is extremely rare. Here we report a case of congenital isolated perineal lipoma presenting as a pseudo-tail and also include a literature review of the condition.