The histologic and immunohistochemical findings of an extremely rare case o
f congenital soft tissue mass on the alveolar ridge in an infant are report
ed. The lesion clinically mimicked an ordinary congenital epulis (congenita
l granular cell epulis, granular cell tumor of the newborn); however, histo
logically it consisted of a conglomerate of spindle-shaped cells, akin to s
mooth muscle cells, which formed interlacing and whorled fasciculi. Nerve f
ibers with myxoid degeneration, capillaries and muscle walled small vessels
intermingled with fasciculi of spindle-shaped cells. The border between th
e conglomerate of spindle-shaped cells and the surrounding connective tissu
e was not evident. Immunohistochemically, most of the spindle-shaped cells
were intensely positive for antibodies to alpha-smooth muscle actin, HHF-35
and desmin. These findings suggest that the lesion was composed of mature
smooth muscle cells that were of hamartomatous or choristomatous nature. Th
e term 'congenital leiomyomatous epulis' is proposed.