Background: Pemphigus is an autoimmune disease characterized with intra-epidermal cleavage. Objective: The aim of this study was to examine the clinical and epidemiologic features of pemphigus in Khouzestan province, southwest Iran. Patients and Methods: All new cases of pemphigus admitted in the department of dermatology, Sina Hospital, Ahwaz University from October 1990 to September 1999 were studied. The diagnosis of pemphigus was based on clinical and histological findings. Results: One hundred-eleven pemphigus patients represented 4.7% of 2358 patients admitted to this department in this period. The most common presentation was pemphigus vulgaris (81.98%), followed by pemphigus foliaceous (18.02%). Pemphigus was observed more common in women (62 of 111; 55.86%) than in men (49 of 111; 44.14%). The mean age of all variants of pemphigus and in both sex at the time of diagnosis was 42.5 years. The lag time between the onset of illness and final diagnosis in pemphigus vulgaris and pemphigus foliaceous were 4 and 9 months, respectively. In pemphigus vulgaris patients, the onset of lesions were 54.65% and 45.34% from mucous membranes (Mainly oral cavity) and skin in 54.65% and 45.35% of cases, respectively. In all of pemphigus foliaceous patients, the onset of lesions was from the skin. The mortality was 6.30%, more in pemphigus foliaceous than vulgaris. Conclusion: This study showed that pemphigus is relatively common in Khouzestan province, compared with other regions. Pemphigus vulgaris is the most common variant and mortality was higher in pemphigus foliaceous.