Perilunate injuries with lunate extrusion are extremely rare, representing an extreme subgroup of the Mayfield type 4 spectrum, with prognosis and management remaining controversial. We present a 45-year-old man with an open type 4 perilunate fracture dislocation, with lunate extrusion through the volar skin. Despite emergent treatment with open reduction and internal fixation, lunate and scaphoid proximal pole avascular necrosis developed. However, the overall functional outcome was satisfactory for daily activities at 30 months follow-up, with substantial improvement in the range of motion after hardware removal. To our knowledge, this is the third reported case of an open perilunate injury with lunate extrusion in the literature.