SMARCB1/INI-1 deficient carcinoma is a rare malignant tumor that seldom occurs in the adrenal gland.A 27-year-old male patient who presented with palpitation and discomfort for 1 week was reported.MRI showed a soft tissue mass in the left adrenal gland with inhomogeneous signal and mild inhomogeneous enhancement of the lesion on enhanced scan.The patient underwent laparoscopic unilateral adrenal gland and mass resection,with postoperative pathology confirming SMARCB1/INI-1 deficient adrenal cancer.A 3-month follow-up showed no evidence of local recurrence or distant metastasis on imaging.