RATIONALE: Nontuberculous mycobacteria infection is becoming more and more common in clinical practice, while skin and soft tissue infection is an important part. The evaluation of the immune status of patients has certain reference value for diagnosis and treatment. PATIENT CONCERNS: A 48-year-old woman developed an erythematosus nodule with purulent discharge on the right hip for 4 months. She had a history of systemic lupus erythematosus for more than 20 years, in stable control with prednisone 10 mg/d, azathioprine 50 mg/12 h, and hydroxychloroquine 200 mg/12 h. There was no trauma prior to the lesion. DIAGNOSES: After excluding other sites involved, the patient was diagnosed as Mycobacterium avium primary cutaneous infection based on laboratory culture, biopsy, and sequencing techniques. INTERVENTIONS: After surgical resection, a combination of oral azithromycin, rifampicin, and ethambutol hydrochloride was given. OUTCOMES: The lesion healed after 4 months with no relapse. LESSONS: Primary cutaneous nontuberculous mycobacteria infection should raise more attention in immunocompromised and even immunocompetent populations.