Background: Connective tissue diseases are defined as a group of auto-immune disorders that have in common, immunological and inflammatory changes in blood vessels and connective tissues. We have undertaken this study to know about the epidemiological and clinical aspects of this disease. Objectives: The present study was conducted to know the occurrence of various types of connective tissue diseases, incidence in relation to gender, the age distribution and the most common symptoms and signs of connective tissue diseases. Methods: The present study was conducted in 40 cases of clinically diagnosed connective tissue diseases in a private hospital in Hyderabad, from April 2014 – April 2015 over a period of 12 months. A detailed history and examination was performed as per proforma. Basic... and appropriate special investigations were performed; including complete haemogram, complete urine examination, renal function tests, skin biopsy and antinuclear antibody positivity test by ELISA was done in all patients.Results: In this study, majority of the patients (17 patients – 42.50 percent) examined were within the age group of 31 – 40 years. Majority of the patients belonged to the female gender (33 patients – 82.50 percent). Discoid Lupus Erythematosus (15 patients – 37.50 percent) was the most common disease which was observed in our study. Photosensitivity (5 patients – 2.50 percent) was the most common presentation in SLE (8 patients – 20.00 percent of all cases). The most common type of morphoea (9 patients – 22.50 percent of all cases) was the plaque type (5 patients – 55.55 cases of morphoea). Sclerosis of the skin (6 patients – 100.00 percent) was the most common finding in systemic sclerosis (15.00 percent of all the cases), followed by Reynaud’s phenomenon (5 patients – 83.33 percent of cases of systemic sclerosis). Antinuclear Antibodies positivity was seen in 28 patients (70.00 percent of all the cases) of connective tissue diseases, but the positivity was highest in systemic lupus erythematosus (7 patients – 87.50 percent of the cases). Conclusion:. Discoid lupus Erythematous being most common presentation. Females of age group 31-40 were most frequently involved.