Mycosis fungoides (MF) represents the most common type of cutaneous lymphoma. The diagnosis of MF may occasionally be challenging, particularly for variants of MF such as folliculotropic MF, syringotropic MF and granulomatous MF. Herein, we describe a case of MF in which syringotropism and a prominent granulomatous reaction were noted around the affected eccrine gland. This case represents a rare example of MF with multiple unusual histopathological features, including syringotropism, a granulomatous reaction and a reactive B-cell proliferation.