Background: Dermatomyositis is an autoimmune inflammatory myopathy with distinct cutaneous findings. Its etiology is unknown. Objective: The purpose of this study was to evaluate demographic, clinical and paraclinical findings in patients with dermatomyositis in Ahwaz. Patients and Methods: This is descriptive, retrospective study and the records of all patients with dermatomyositis admitted to Sina hospital in Ahwaz from December 1990 to March 2000 analyzed. Results: 10 cases with dermatomyositis were admitted during the time. The ages of 5 patients were less than 16 years and 5 were over twenty years. Nine of the patients were female and 1 was male. Heliotrope rash was the first cutaneous sign in 9 and 6 patients had Gottron’s papules. All of the patients had proximal muscle myopathy and 1 patient also had nasopharyngeal involvement. The most common abnormal laboratory tests were the rise of SGOT and CPK. EMG were done on 9 patients and all had diagnostic findings. 1 patient was pregnant and she also had pneumonia and perianal abscess. In 1 patient wound healing was impaired. None of patients had malignancy. Conclusion: Dermatomyositis is more common in women then in men and the most common sign of the disease is heliotrope rash in Ahwaz.