Background A substantial number of deaths and morbidities are caused by pulmonary involvement in connective tissue disorders (CTDs). The majority of patients suffering from CTDs show diverse clinical presentations arising in the lungs, the diaphragm, vasculature, and the pleura during the course of their disease. The clinical course is highly variable ranging from asymptomatic to symptomatic and from reversible to an irreversible stage. The most common occurring pulmonary appearance is the interstitial disease of the lung. In our study, we aim to determine the prevalence and pattern of pulmonary manifestation in patients with CTD presenting at a tertiary care hospital of Karachi, Pakistan. Methods A descriptive cross-sectional study was conducted in the department of Rheumatology Medical Unit II, Jinnah Postgraduate Medical Centre (JPMC), Karachi, Pakistan. All patients having CTD were enrolled. Patients with human immunodeficiency virus (HIV) infection, smokers, chronic obstructive pulmonary disease (COPD), bronchogenic carcinoma and patients with underlying cardiac disease were excluded from the study. Pulmonary manifestations like nonspecific interstitial pneumonia (NSIP), pleural effusion, usual interstitial pneumonia (UIP), interstitial pneumonitis, pulmonary arterial hypertension (PAH), tuberculosis, and pulmonary embolism were observed. In addition to this, treatment history and immunological test findings of these patients were also recorded. Results A total of 290 patients with rheumatologic disorders were included in this study, out of which 90.7% (263) were females and the average age of the entire cohort was 35.13 ± 12.53 years. A majority, 71% (206), of the patients were young (≤ 40 years of age). Rheumatoid arthritis (RA) was the most commonly observed rheumatologic disorder in this cohort which was in 70.3% (204) patients. RA was more prevalent in patients above 40 years of age as compared to young patients (≤ 40 years of age) with a frequency of 81% vs. 66%; p=0.012. Pulmonary involvement was observed in 27.2% of the patients while no pulmonary involvement was noted in 72.8% of patients. Conclusion Pulmonary manifestations in patients with CTD is not that uncommon (27.2%). PAH and NSIP collectively account for three-fourth of the total prevalent cases of pulmonary involvement in CTD patients.