The differential diagnosis of pyrexia, lung nodules and granulomas includes tuberculosis, vasculitis and rarely a malignancy. In countries where tuberculosis or histoplasmosis is endemic, these are the first consideration and often ruled out by microbiological investigations. Vasculitis like granulomatosis with polyangitis (Wegener's granulomatosis), Churg strauss syndrome and sarcoidosis, which are the second consideration, are ruled out by serological investigations. Confirmation of malignancy merits histopathology. This case highlights how a rare diagnosis of pulmonary lymphomatoid granulomatosis was reached after an open lung biopsy. The following case also describes the natural history of this rare disease as it showed transient spontaneous remission but ultimately required therapy.