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Introduction Prader‐Willi syndrome is a congenital disorder that occurs in one in 10 000–30 000 children and is characterized by obesity, short stature, and intellectual disability. Case presentation A 24‐year‐old male patient with Prader‐Willi syndrome presented with an enlarged adrenal tumor. Computed tomography detected a well‐defined mass. Magnetic resonance imaging revealed an increased signal intensity predominantly in fatty areas, suggesting adrenal myelolipoma. Laparoscopic left adrenalectomy was performed. Postoperatively, the patient developed mild pulmonary atelectasis, myelolipoma was confirmed by histopathology, and there was no recurrence at approximately 2 years postoperatively. Conclusion This is the first report of Prader‐Willi syndrome complicated with adrenal myelolipoma, which was removed laparoscopically.